[vc_row full_width=”stretch_row” content_placement=”top” woodmart_bg_position=”right-top” row_reverse_mobile=”1″ row_reverse_tablet=”1″ woodmart_disable_overflow=”1″ css=”.vc_custom_1539348987196{padding-top: 8vh !important;padding-bottom: 4vh !important;background-image: url(https://aiun.ltd/wp-content/uploads/2019/12/motorcycle-hotspot-background.jpg) !important;background-position: 0 0 !important;background-repeat: no-repeat !important;}” woodmart_parallax=”0″ woodmart_gradient_switch=”no”][vc_column css=”.vc_custom_1535720256088{margin-bottom: 4vh !important;padding-top: 0px !important;}” offset=”vc_col-lg-8 vc_col-md-7″][woodmart_image_hotspot img=”4945″ img_size=”full”][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”53.358||88.164″ title=”Tumor necrosis factor alpha or TNFα” link_text=”https://aiun.ltd/immune-system-components/tumor-necrosis-factor-alpha-or-tnf%ce%b1/”]Tumor necrosis factor (TNF, cachexin, or cachectin; once named as tumor necrosis factor alpha or TNFα) is a cell signaling protein (cytokine) involved in systemic inflammation and is one of the cytokines that make up the acute phase reaction. It is produced chiefly by activated macrophages, although it can be produced by many other cell types such as CD4+ lymphocytes, NK cells, neutrophils, mast cells, eosinophils, and neurons.[5] TNF is a member of the TNF superfamily, consisting of various transmembrane proteins with a homologous TNF domain.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”41.304||68.675″ title=”ICAM-1″ link=”url:https%3A%2F%2Faiun.ltd%2Ficam-1%2F|title:ICAM-1||”]ICAM-1 also known as CD54 is a protein that in humans is encoded by the ICAM1 gene. This gene encodes a cell surface glycoprotein which is typically expressed on endothelial cells and cells of the immune system.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”41.486||57.349″ title=”VCAM-1″ link_text=”https://aiun.ltd/vcam-1/”]Vascular cell adhesion protein 1 also known as vascular cell adhesion molecule 1 or cluster of differentiation 106 is a protein that in humans is encoded by the VCAM1 gene. VCAM-1 functions as a cell adhesion molecule[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”25.725||66.024″ title=”LFA-1″ link_text=”https://aiun.ltd/lfa-1/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”26.268||74.217″ title=”VLA-4″ link_text=”https://aiun.ltd/vla-4/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”26.087||82.410″ title=”MaC-1″ link_text=”https://aiun.ltd/mac-1/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”11.413||89.880″ title=”Chemokines” link_text=”https://aiun.ltd/chemokines/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”81.341||63.373″ title=”STAT 1″ link_text=”https://aiun.ltd/stat-1/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”80.978||67.229″ title=”MHC-1″][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”60.688||84.337″ title=”Nitric Oxide”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”85.688||74.458″ title=”Cathespin”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”84.783||77.349″ title=”HSP 70-90″][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”82.428||84.337″ title=”NCAM”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”86.413||91.566″ title=”MxA”]The human MxA protein is part of the antiviral state induced by alpha/beta interferon MxA inhibits the multiplication of several RNA viruses in cell[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”5.435||58.795″ title=”Cytokines”]Cytokines are a broad and loose category of small proteins that are important in cell signaling. Cytokines are peptides, and cannot cross the lipid bilayer of cells to enter the cytoplasm. Cytokines have been shown to be involved in autocrine, paracrine and endocrine signaling as immunomodulating agents.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”MAC”]The membrane-attack complex (MAC) forms transmembrane channels. These channels disrupt the cell membrane of target cells, leading to cell lysis and death.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”35.688||48.675″ title=”M1-2″]Mi-2/NuRD (Nucleosome Remodeling Deacetylase) complex, is a group of associated proteins with both ATP-dependent chromatin remodeling and histone deacetylase activities[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”C3b NEO” hotspot=”46.377||39.759″]The MAC and the immune complex-specific C3bNEO fragment are deposited on the endomysial capillaries early in the disease and lead sequentially to loss of capillaries, muscle ischemia, muscle fiber necrosis, and perifascicular atrophy.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”C3a” hotspot=”7.790||48.193″]C3a is a 77 residue anaphylatoxin that binds to the C3a receptor (C3aR), a class A G protein-coupled receptor. It plays a large role in the immune response.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”TGF-b”]Transforming growth factor beta is a multifunctional cytokine belonging to the transforming growth factor superfamily that includes three different mammalian isoforms and many other signaling proteins.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”52.717||71.566″ title=”B cells”]B cells, also known as B lymphocytes, are a type of white blood cell of the small lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system by secreting antibodies[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”79.710||88.193″ title=”Mi-1″]The Mi-2/NuRD (Nucleosome Remodeling Deacetylase) complex, is a group of associated proteins with both ATP-dependent chromatin remodeling and histone deacetylase activities.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”53.442||22.892″ title=”Endothelial cell wall”]Endothelium refers to cells that line the interior surface of blood vessels and lymphatic vessels, forming an interface between circulating blood or lymph in the lumen and the rest of the vessel wall.[/woodmart_hotspot][/woodmart_image_hotspot][vc_empty_space height=”6vh” woodmart_hide_large=”0″ woodmart_hide_medium=”0″ woodmart_hide_small=”0″ woodmart_hide_extra_small=”0″][/vc_column][vc_column css=”.vc_custom_1535720261744{margin-bottom: 4vh !important;padding-top: 0px !important;}” offset=”vc_col-lg-4 vc_col-md-5 vc_col-xs-12″][woodmart_responsive_text_block size=”custom” font_weight=”600″ color_scheme=”light” align=”left” css=”.vc_custom_1539595667890{margin-bottom: 15px !important;}” woodmart_css_id=”5bc45d8c5aadf” text_font_size=”eyJwYXJhbV90eXBlIjoid29vZG1hcnRfcmVzcG9uc2l2ZV9zaXplIiwiY3NzX2FyZ3MiOnsiZm9udC1zaXplIjpbIiAud29vZG1hcnQtdGV4dC1ibG9jayJdfSwic2VsZWN0b3JfaWQiOiI1YmM0NWQ4YzVhYWRmIiwiZGF0YSI6eyJkZXNrdG9wIjoiNDVweCIsInRhYmxldCI6IjMycHgiLCJtb2JpbGUiOiIyOHB4In19″ content_width=”100″]Together
This Affords a Sporty Position.[/woodmart_responsive_text_block][woodmart_responsive_text_block font=”text” size=”custom” color_scheme=”light” align=”left” desktop_text_size=”14″ css=”.vc_custom_1536053818138{margin-bottom: 25px !important;}”]Nor again is there anyone who loves or pursues or desires to obtain pain of itself, because it is pain, but because occasionally circumstances toil.[/woodmart_responsive_text_block][woodmart_button color="primary" shape="semi-round" align="left" title="View More" link="url:%23|||"][/vc_column][/vc_row][vc_row css=”.vc_custom_1540209170412{margin-right: -10px !important;margin-left: -10px !important;}” woodmart_parallax=”0″ woodmart_gradient_switch=”no” row_reverse_mobile=”0″ row_reverse_tablet=”0″ woodmart_disable_overflow=”0″][vc_column css=”.vc_custom_1539349812585{margin-top: -8vh !important;margin-bottom: 6vh !important;padding-top: 0px !important;}” woodmart_parallax=”0″ parallax_scroll=”no” woodmart_sticky_column=”false”][vc_row_inner][vc_column_inner width=”1/2″ css=”.vc_custom_1539352179865{margin-bottom: 20px !important;padding-top: 0px !important;padding-right: 10px !important;padding-left: 10px !important;}” offset=”vc_col-lg-4 vc_col-md-4 vc_col-xs-12″][vc_video align=”center” poster_image=”3646″ image_poster_switch=”yes” img_size=”full” css=”.vc_custom_1539343713801{margin-bottom: 0px !important;}”][/vc_column_inner][vc_column_inner width=”1/2″ css=”.vc_custom_1539352192339{margin-bottom: 20px !important;padding-top: 0px !important;padding-right: 10px !important;padding-left: 10px !important;}” offset=”vc_col-lg-4 vc_col-md-4 vc_col-xs-12″][vc_video align=”center” poster_image=”3646″ image_poster_switch=”yes” img_size=”full” css=”.vc_custom_1539343722747{margin-bottom: 0px !important;}”][/vc_column_inner][vc_column_inner width=”1/3″ css=”.vc_custom_1539352202225{margin-bottom: 20px !important;padding-top: 0px !important;padding-right: 10px !important;padding-left: 10px !important;}” offset=”vc_col-lg-4 vc_col-md-4 vc_hidden-sm vc_col-xs-12″][vc_video align=”center” poster_image=”3646″ image_poster_switch=”yes” img_size=”full” css=”.vc_custom_1539343731880{margin-bottom: 0px !important;}”][/vc_column_inner][/vc_row_inner][/vc_column][/vc_row][vc_row][vc_column][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Dermatomyositis may be associated with celiac disease even in the absence of gastrointestinal symptoms.” tab_id=”1581776528917-f0335080-cdfa”][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][/vc_column][/vc_row][vc_row][vc_column][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Lupus erythematosus and other autoimmune diseases related to statin therapy have been reported.” tab_id=”1581777190800-9218a065-0dad”][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][/vc_column][/vc_row][vc_row][vc_column][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis” tab_id=”1581778397246-4b0ef946-0592″][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][/vc_column][/vc_row][vc_row][vc_column][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Tobacco smoking was associated with clinical and autoantibody phenotypes in Caucasians.” tab_id=”1581778721968-55668ac2-f74e”][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”A dermatomyositis-like syndrome has been linked to statin drug use.” tab_id=”1581778724103-7e8083ca-0a21″][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Creatine supplements combined with home exercises improve functional performance without significant adverse effects in patients with polymyositis or dermatomyositis.” tab_id=”1581778867862-eabb7c70-1bc6″][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Fluvastatin-induced dermatomyositis has been reported.” tab_id=”1581779277876-e0774473-39ba”][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Hepatitis B vaccine associated with dermatomyositis has been reported.” tab_id=”1581779431459-844f61df-47a9″][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Sodium Thiosulfate for Treatment of Calcinosis Associated With Juvenile and Adult Dermatomyositis” tab_id=”1581779824642-73d09535-1b58″][vc_column_text text_larger=”no”]
[/vc_column_text][/vc_tta_section][/vc_tta_accordion][/vc_column][/vc_row][vc_row full_width=”stretch_row” content_placement=”middle” woodmart_disable_overflow=”1″ css=”.vc_custom_1535543746090{margin-bottom: 8vh !important;padding-top: 8vh !important;padding-bottom: 4vh !important;background-image: url(https://aiun.ltd/wp-content/uploads/2019/12/wood-main-bg.jpg) !important;background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}”][vc_column css=”.vc_custom_1530611754151{padding-top: 0px !important;}”][woodmart_responsive_text_block size=”custom” font_weight=”600″ color_scheme=”dark” content_width=”50″ css=”.vc_custom_1581781702522{margin-bottom: 35px !important;}” woodmart_css_id=”5e4812b24d599″ text_font_size=”eyJwYXJhbV90eXBlIjoid29vZG1hcnRfcmVzcG9uc2l2ZV9zaXplIiwiY3NzX2FyZ3MiOnsiZm9udC1zaXplIjpbIiAud29vZG1hcnQtdGV4dC1ibG9jayJdfSwic2VsZWN0b3JfaWQiOiI1ZTQ4MTJiMjRkNTk5IiwiZGF0YSI6eyJkZXNrdG9wIjoiNjRweCIsInRhYmxldCI6IjM4cHgiLCJtb2JpbGUiOiIzMnB4In19″ inline=”no”]Dermatomyositis cell pathogenesis.[/woodmart_responsive_text_block][/vc_column][vc_column width=”2/3″ offset=”vc_col-sm-offset-2″ css=”.vc_custom_1533116005031{padding-top: 0px !important;}” el_class=”z-index-100″][woodmart_image_hotspot img=”4998″ img_size=”full”][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”53.261||3.441″ title=”Antigen” link_text=”Antigen” link=”url:https%3A%2F%2Faiun.ltd%2Fantigen%2F|title:Antigen|target:%20_blank|”]A toxin or other foreign substance which induces an immune response in the body, especially the production of antibodies.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”88.225||22.267″ title=”Clonal expansion” link_text=”Clonal expansion” link=”url:https%3A%2F%2Faiun.ltd%2Fclonal-expansion%2F|title:Clonal%20expansion%C2%A0||”]
[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”48.370||17.611″ title=”MCH” link_text=”MCH” link=”url:https%3A%2F%2Faiun.ltd%2Fmajor-histocompatibility-complex-mhc%2F|||”]The MHC molecules are glycoproteins encoded in a large cluster of genes located on chromosome 6. They were first identified by their potent effect on the immune response to transplanted tissue
The principal function of the MHC is to present antigen to T cells to discriminate between self (our cells and tissues) and nonself (the invaders or modified self).[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”top” hotspot=”10.145||65.789″ title=”T-cell receptor (TCR)” link_text=”T-cell receptor (TCR)” link=”url:https%3A%2F%2Faiun.ltd%2Ft-cell-receptor-tcr%2F|title:T-cell%20receptor%C2%A0(TCR)||”]The T-cell receptor (TCR) is a molecule found on the surface of T cells, or T lymphocytes, that is responsible for recognizing fragments of antigen as peptides bound to major histocompatibility complex (MHC) molecules.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”22.464||61.943″ title=”CD28 (Cluster of Differentiation 28)” link_text=”CD28″ link=”url:https%3A%2F%2Faiun.ltd%2Fcd28-cluster-of-differentiation-28%2F|title:CD28|target:%20_blank|”]CD28 (Cluster of Differentiation 28) is one of the proteins expressed on T cells that provide co-stimulatory signals required for T cell activation and survival. T cell stimulation through CD28 in addition to the T-cell receptor (TCR) can provide a potent signal for the production of various interleukins (IL-6 in particular).[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”34.420||61.943″ title=”CTLA-4″ link_text=”CTLA-4″ link=”url:https%3A%2F%2Faiun.ltd%2Fctla-4%2F|title:CTLA-4%C2%A0||”]CTLA4 or CTLA-4 (cytotoxic T-lymphocyte-associated protein 4), also known as CD152 (cluster of differentiation 152), is a protein receptor that functions as an immune checkpoint and downregulates immune responses.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”50.725||47.166″ title=”CD8″ link_text=”CD8″ link=”url:https%3A%2F%2Faiun.ltd%2Fcd8%2F|title:CD8|target:%20_blank|”]CD8 (cluster of differentiation 8) is a transmembrane glycoprotein that serves as a co-receptor for the T cell receptor (TCR).[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”34.420||38.866″ title=”Integrins” link_text=”Integrins” link=”url:https%3A%2F%2Faiun.ltd%2Fintegrins%2F|title:Integrins%C2%A0|target:%20_blank|”]Integrins are transmembrane receptors that facilitate cell-extracellular matrix (ECM) adhesion. Upon ligand binding, integrins activate signal transduction pathways that mediate cellular signals such as regulation of the cell cycle, organization of the intracellular cytoskeleton, and movement of new receptors to the cell membrane. The presence of integrins allows rapid and flexible responses to events at the cell surface (e.g. signal platelets to initiate an interaction with coagulation factors).
Several types of integrins exist, and one cell may have multiple different types on its surface. Integrins are found in all animals while integrin-like receptors are found in plant cells.
Integrins work alongside other proteins such as cadherins, the immunoglobulin superfamily cell adhesion molecules, selectins and syndecans, to mediate cell–cell and cell–matrix interaction. Ligands for integrins include fibronectin, vitronectin, collagen and laminin.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”83.877||45.142″ title=”MMPs” link_text=”MMPs” link=”url:https%3A%2F%2Faiun.ltd%2Fmatrix-metalloproteinases%2F|title:MMPs%C2%A0|target:%20_blank|”]Matrix metalloproteinases (MMPs), the main extracellular matrix (ECM) enzymes in collagen degradation, as a target for anticancer drugs.
The MMPs play an important role in tissue remodeling associated with various physiological or pathological processes such as morphogenesis, angiogenesis, tissue repair, cirrhosis, arthritis, and metastasis.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”51.630||58.502″ title=”LFA-1″ link_text=”LFA-1″ link=”url:https%3A%2F%2Faiun.ltd%2Flymphocyte-function-associated-antigen-1-lfa-1%2F|title:LFA-1|target:%20_blank|”]Lymphocyte function-associated antigen 1 (LFA-1) is an integrin found on lymphocytes and other leukocytes.[1] LFA-1 plays a key role in emigration, which is the process by which leukocytes leave the bloodstream to enter the tissues. LFA-1 also mediates firm arrest of leukocytes.[2] Additionally, LFA-1 is involved in the process of cytotoxic T cell mediated killing as well as antibody mediated killing by granulocytes and monocytes.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”65.036||59.109″ title=”MMP-9″ link_text=”MMP-9″ link=”url:https%3A%2F%2Faiun.ltd%2Fmmp-9%2F|title:MMP-9|target:%20_blank|”]Matrix metallopeptidase 9 (MMP-9), also known as 92 kDa type IV collagenase, 92 kDa gelatinase or gelatinase B (GELB), is a matrixin, a class of enzymes that belong to the zinc-metalloproteinases family involved in the degradation of the extracellular matrix.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”13.587||7.692″ title=”Macrophage” link_text=”Macrophage” link=”url:https%3A%2F%2Faiun.ltd%2Fmacrophage%2F|title:macrophage|target:%20_blank|”]A macrophage is a type of phagocyte, which is a cell responsible for detecting, engulfing and destroying pathogens and apoptotic cells. Macrophages are produced through the differentiation of monocytes, which turn into macrophages when they leave the blood.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”60.507||53.846″ title=”IFNγ” link_text=”IFNγ” link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5033%26preview%3Dtrue|title:IFN%CE%B3|target:%20_blank|”]Interferon gamma (IFNγ) is a dimerized soluble cytokine that is the only member of the type II class of interferons. The existence of this interferon, which early in its history was known as immune interferon, was described by E. F. Wheelock as a product of human leukocytes stimulated with phytohemagglutinin, and by others as a product of antigen-stimulated lymphocytes.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”80.072||57.895″ title=”IL-2″ link_text=”IL-2″ link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5034%26preview%3Dtrue|title:IL-2|target:%20_blank|”]Interleukin-2 (IL-2) is an interleukin, a type of cytokine signaling molecule in the immune system. It is a 15.5 – 16 kDa protein that regulates the activities of white blood cells (leukocytes, often lymphocytes) that are responsible for immunity. IL-2 is part of the body’s natural response to microbial infection, and in discriminating between foreign (“non-self”) and “self”. IL-2 mediates its effects by binding to IL-2 receptors, which are expressed by lymphocytes. The major sources of IL-2 are activated CD4+ T cells and activated CD8+ T cells.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”63.225||73.684″ title=”Perforin” link_text=”Perforin” link=”url:https%3A%2F%2Faiun.ltd%2Fperforin%2F|title:Perforin|target:%20_blank|”]Perforin is a pore forming cytolytic protein found in the granules of cytotoxic T lymphocytes (CTLs) and natural killer cells (NK cells). Upon degranulation, perforin binds to the target cell’s plasma membrane, and oligomerises in a Ca2+ dependent manner to form pores on the target cell.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”29.529||41.903″ title=”Chemokines” link_text=”Chemokines” link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5038%26preview%3Dtrue|title:Chemokines%C2%A0|target:%20_blank|”]Chemokines (Greek -kinos, movement) are a family of small cytokines, or signaling proteins secreted by cells. Their name is derived from their ability to induce directed chemotaxis in nearby responsive cells; they are chemotactic cytokines.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”87.862||87.045″ title=”Necrosis” link_text=”Necrosis” link=”url:https%3A%2F%2Faiun.ltd%2Fnecrosis%2F|title:Necrosis%C2%A0|target:%20_blank|”]
[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”33.877||70.445″ title=”BB1″ link_text=”BB1 monoclonal antibodies”]BB1 monoclonal antibodies[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”57.246||72.672″ title=”MMP-2″ link_text=”MMP-2″ link=”url:https%3A%2F%2Faiun.ltd%2Fmmp-2%2F|title:MMP-2|target:%20_blank|”]72 kDa type IV collagenase also known as matrix metalloproteinase-2 (MMP-2) and gelatinase A is an enzyme that in humans is encoded by the MMP2 gene.[5] The MMP2 gene is located on chromosome 16 at position 12.2[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”42.572||94.130″ img_size=”TAP” link_text=”TAP” link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5049%26preview%3Dtrue|title:TAP|target:%20_blank|”]Transporter associated with antigen processing (TAP) protein complex belongs to the ATP-binding-cassette transporter family.[1] It delivers cytosolic peptides into the endoplasmic reticulum (ER), where they bind to nascent MHC class I molecules[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”31.703||97.773″ title=”ER” link_text=”ER” link=”url:https%3A%2F%2Faiun.ltd%2Fthe-endoplasmic-reticulum-er%2F|title:ER|target:%20_blank|”]The endoplasmic reticulum (ER) is a type of organelle made up of two subunits – rough endoplasmic reticulum (RER), and smooth endoplasmic reticulum (SER). The endoplasmic reticulum is found in most eukaryotic cells and forms an interconnected network of flattened, membrane-enclosed sacs known as cisternae (in the RER), and tubular structures in the SER. The membranes of the ER are continuous with the outer nuclear membrane. The endoplasmic reticulum is not found in red blood cells, or spermatozoa.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”56.341||77.126″ title=”Calnexin” link_text=”Calnexin” link=”url:https%3A%2F%2Faiun.ltd%2Fcalnexin%2F|title:Calnexin|target:%20_blank|”]Calnexin is a chaperone, characterized by assisting protein folding and quality control, ensuring that only properly folded and assembled proteins proceed further along the secretory pathway. It specifically acts to retain unfolded or unassembled N-linked glycoproteins in the ER.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”73.188||60.931″ title=”IL-1α” link_text=”IL-1α” link=”url:https%3A%2F%2Faiun.ltd%2Finterleukin-1-alpha-il-1%25ce%25b1%2F|title:IL-1%CE%B1|target:%20_blank|”]Interleukin 1 alpha (IL-1α) also known as hematopoietin 1 is a cytokine of the interleukin 1 family that in humans is encoded by the IL1A gene. In general, Interleukin 1 is responsible for the production of inflammation, as well as the promotion of fever and sepsis. IL-1α inhibitors are being developed to interrupt those processes and treat diseases.[/woodmart_hotspot][woodmart_hotspot][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”22.464||47.773″ title=”MIG” link_text=”MIG” link=”url:https%3A%2F%2Faiun.ltd%2Fmig%2F|title:MIG|target:%20_blank|”]MIG, a member of the CXC subfamily of chemokines is an inflammatory chemokine that is important in the recruitment of activated T-cells to sites of infection. MIG enhances Th1 and Th2 polarization, attracting Th1 cells and inhibiting Th2 migration.[/woodmart_hotspot][/woodmart_image_hotspot][/vc_column][/vc_row][vc_row full_width=”stretch_row” content_placement=”middle” woodmart_disable_overflow=”1″ css=”.vc_custom_1535543746090{margin-bottom: 8vh !important;padding-top: 8vh !important;padding-bottom: 4vh !important;background-image: url(https://aiun.ltd/wp-content/uploads/2019/12/wood-main-bg.jpg) !important;background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}”][vc_column css=”.vc_custom_1530611754151{padding-top: 0px !important;}”][woodmart_responsive_text_block size=”custom” font_weight=”600″ color_scheme=”dark” content_width=”50″ desktop_text_size=”64″ tablet_text_size=”38″ mobile_text_size=”32″ css=”.vc_custom_1530782250887{margin-bottom: 35px !important;}”]The Best Choice For A Winter Ride.[/woodmart_responsive_text_block][/vc_column][vc_column width=”2/3″ offset=”vc_col-sm-offset-2″ css=”.vc_custom_1533116005031{padding-top: 0px !important;}” el_class=”z-index-100″][woodmart_image_hotspot img=”5853″ woodmart_color_scheme=”light” img_size=”full”][woodmart_hotspot hotspot_dropdown_side=”right” hotspot=”85.413||37.684″ product_id=”53″][/woodmart_hotspot][woodmart_hotspot hotspot_dropdown_side=”right” hotspot=”58.509||6.985″ product_id=”53″][/woodmart_hotspot][woodmart_hotspot hotspot_dropdown_side=”right” hotspot=”33.874||34.926″ product_id=”53″][/woodmart_hotspot][woodmart_hotspot hotspot_dropdown_side=”top” hotspot=”11.507||62.684″ product_id=”53″][/woodmart_hotspot][/woodmart_image_hotspot][/vc_column][/vc_row]
Abstract Title:
Dermatomyositis associated with celiac disease: response to a gluten-free diet.
Abstract Source:
Can J Gastroenterol. 2006 Jun;20(6):433-5. PMID: 16779462
Abstract:
The association between dermatomyositis and celiac disease in children has been well documented. In the adult population, however, the association has not been clearly established. A rare case of concomitant dermatomyositis and celiac disease in a 40-year-old woman is presented. After having been diagnosed with dermatomyositis and iron deficiency anemia, this patient was referred to the gastroenterology clinic to exclude a gastrointestinal malignancy. Blood tests revealed various vitamin deficiencies consistent with malabsorption. The results of gastroscopy with duodenal biopsy were consistent with celiac disease. After she was put on a strict gluten-free diet, both nutritional deficiencies and the dermatomyositis resolved. The patient’s human leukocyte antigen haplotype study was positive for DR3 and DQ2, which have been shown to be associated with both juvenile dermatomyositis and celiac disease. It is suggested that patients with newly diagnosed dermatomyositis be investigated for concomitant celiac disease even in the absence of gastrointestinal symptoms.
Article Published Date : Jun 01, 2006
Study Type : Human Study
Abstract Title:
Lupus erythematosus and other autoimmune diseases related to statin therapy: a systematic review.
Abstract Source:
J Eur Acad Dermatol Venereol. 2007 Jan ;21(1):17-24. PMID: 17207162
Article Affiliation:
Department of Dermatology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. bernard.noel@chuv.hospvd.ch
Abstract:
BACKGROUND: Statins have been increasingly associated with drug-induced autoimmune reactions, including lupus erythematosus.
OBJECTIVE: To identify and determine the clinical and biological characteristics of statin-induced autoimmune reactions.
MATERIAL AND METHODS: The MEDLINE database (1966 to September 2005) was used to identify all reported cases of statin-induced autoimmune diseases. The keywords used were statins, 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors, adverse effects, autoimmune disease, lupus erythematosus, dermatomyositis and polymyositis.
RESULTS: Twenty-eight cases of statin-induced autoimmune diseases have been published so far. Systemic lupus erythematosus was reported in 10 cases, subacute cutaneous lupus erythematosus in three cases, dermatomyositis and polymyositis in 14 cases and lichen planus pemphigoides in one case. Autoimmune hepatitis was observed in two patients with systemic lupus erythematosus. The mean time of exposure before disease onset was 12.8+/-18 months; range 1 month-6 years. Systemic immunosuppressive therapy was required in the majority of cases. In many patients, antinuclear antibodies were still positive many months after clinical recovery. A lethal outcome has been recorded in two patients despite aggressive immunosuppressive therapy.
CONCLUSION: Long-term exposure to statins may be associated with drug-induced lupus erythematosus and other autoimmune disorders. Fatal cases have been reported despite early drug discontinuation and aggressive systemic immunosuppressive therapy.
Article Published Date : Jan 01, 2007
Study Type : Human Study
PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
About this article
Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
- Published
- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Abstract Title:
The effect of cigarette smoking on the clinical and serological phenotypes of polymyositis and dermatomyositis.
Abstract Source:
Semin Arthritis Rheum. 2018 Feb 14. Epub 2018 Feb 14. PMID: 29703532
Article Affiliation:
Adam Schiffenbauer
Abstract:
OBJECTIVE: Cigarette smoking is associated with immune-mediated disorders. We explored the contribution of smoking to polymyositis (PM) and dermatomyositis (DM) phenotypes and attempted to determine whether cigarette smoking effects differ by race and genotype.
METHODS: Associations of tobacco smoking with disease features, autoantibodies, HLA types, and race were evaluated using multiple logistic regressions in 465 patients.
RESULTS: Caucasian ever-smokers (n = 140) were more likely to have PM (adjusted OR = 2.24, 95% CI: 1.41-3.57), anti-synthetase (adjusted OR = 1.93, 95% CI: 1.12-3.34) and anti-Jo-1 autoantibodies (adjusted OR = 1.94, 95% CI: 1.08-3.46) and less likely to have anti-p155/140 autoantibodies (adjusted OR = 0.36, 95% CI: 0.14-0.92). In Caucasians, ever-smokers had a greater interstitial lung disease (ILD) frequency than never-smokers, while in African-Americans this relationship was inverted, but neither trend reached statistical significance. Pack-years of cigarette smoking showed significant positive associations with PM (adjusted OR = 1.02, 95% CI: 1.002-1.04) and ILD (adjusted OR = 1.02, 95% CI: 1.001-1.03) and was inversely associated with anti-p155/140 autoantibodies (adjusted OR = 0.93, 95% CI: 0.87-0.99) in Caucasians. Caucasian heavy smokers (≥20 pack-years) were more likely to have PM (adjusted OR = 2.52, 95% CI: 1.25-5.09), ILD (adjusted OR = 2.48, 95% CI: 1.23-5.00) and anti-Jo-1 autoantibodies (adjusted OR = 2.65, 95% CI: 1.16-6.08) than never-smokers. In Caucasians, compared to never-smokers without HLA-DRB1*03:01 allele, ever-smokers with HLA-DRB1*03:01 allele had the highest odds of PM, ILD, ASA, and anti-Jo-1 autoantibodies. Risks for those with only one of these two factors were intermediate. An inverse pattern was observed regarding anti-p155/140 autoantibodies.
CONCLUSION: Tobacco smoking was associated with clinical and autoantibody phenotypes in Caucasians. Our findings also suggest possible interactions among HLA-DRB1*03:01 and smoking on the risk of PM and ILD, as well as, anti-synthetase, anti-Jo-1, and anti-p155/140 autoantibodies in Caucasians.
Article Published Date : Feb 13, 2018
Study Type : Human Study
Abstract Title:
Dermatomyositis-like syndrome and HMG-CoA reductase inhibitor (statin) intake.
Abstract Source:
Muscle Nerve. 2004 Dec ;30(6):803-7. PMID: 15389654
Article Affiliation:
Department of Neurology, Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA.
Abstract:
A patient developed an adult-onset dermatomyositis-like syndrome characterized by skin rash and progressive proximal muscle weakness concurrent with the intake of simvastatin. Despite discontinuation of the statin, symptoms progressed and required conventional steroid therapy for remission. The association between statins and the development of a musculocutaneous syndrome closely resembling dermatomyositis in susceptible subjects is poorly understood and has been reported rarely. The purpose of this report is to provide additional support for this pathological association. Since the population receiving statins is large and rapidly growing, caregivers are urged to be alert regarding the early recognition and proper care of the spectrum of neuromuscular complications linked to statin intake.
Article Published Date : Dec 01, 2004
Study Type : Human: Case Report
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
About this article
Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
- Published
- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Creatine supplements in patients with idiopathic inflammatory myopathies who are clinically weak after conventional pharmacologic treatment: Six-month, double-blind, randomized, placebo-controlled trial
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
About this article
Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
- Published
- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Abstract Title:
[Fluvastatin-induced dermatomyositis].
Abstract Source:
Ann Dermatol Venereol. 2005 Dec ;132(12 Pt 1):996-9. PMID: 16446645
Article Affiliation:
Service de Dermatologie, Centre Hospitalier Universitaire de Caen.
Abstract:
BACKGROUND: Dermatomyositis is a rare inflammatory dermatosis for which an iatrogenic origin has been described in very few cases. We report a case of dermatomyositis occurring after fluvastatin intake.
CASE REPORT: A 76-year-old male patient sought medical attention for a photodistributed rash and considerable muscular weakness present for one month. Two months earlier, fluvastatin had been introduced following the discovery of dyslipidemia. Serum creatine phosphokinase levels were elevated. Histological examination of a muscle biopsy was consistent with a diagnosis of dermatomyositis. Investigation for neoplasia and associated autoimmune disease proved negative. All clinical and laboratory abnormalities diminished spontaneously without recourse to corticosteroids within one month of the final intake of fluvastatin. After a follow-up period of three years, no recurrence was observed and no signs of neoplasia or connectivitis were found.
DISCUSSION: Iatrogenic dermatomyositis has only been reported in rare cases. Certain drugs have been incriminated, notably D-penicillamine. Six cases of drug-induced dermatomyositis have been described with statins: simvastatin, atorvastatin, pravastatin and lovastatin. Of these cases, only one resolved spontaneously after withdrawal of the drug alone without use of corticosteroids. Our case concerns intake of fluvastatin, an HMG-CoA reductase inhibitor with rare though well-known muscular side effects: elevated serum CPK, myalgia and rhabdomyolysis. Six cases of polymyositis have also been reported. Ours is the first case of dermatomyositis described with this category of statins. It is also the second reported case showing improvement after withdrawal of the lipid-lowering agent and without corticosteroids. Thus in cases of dermatomyositis, this iatrogenic picture should be sought routinely.
Article Published Date : Dec 01, 2005
Study Type : Human: Case Report
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
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Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
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- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Abstract Title:
HBV vaccine and dermatomyositis: is there an association?
Abstract Source:
Rheumatol Int. 2008 Apr;28(6):609-12. Epub 2007 Nov 23. PMID: 18034245
Article Affiliation:
Center for Autoimmune Diseases and Department of Medicine B, Sheba Tel-Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Abstract:
The etiology of dermatomyositis is unknown, but immune mechanisms play an important role. Several dermatological manifestations have been reported among carriers of hepatitis B surface antigen, and after vaccination with the HBV vaccine. Almost all the skin reactions described were peculiar skin eruptions suggestive of an immune complex reaction. Some authors described the occurrence of dermatomyositis after BCG and influenza vaccination. We report a case of a 6-year-old child, who was vaccinated for hepatitis B virus and developed a flu-like disease accompanied by a skin rash, which had the typical features of dermatomyositis. The association of vaccination with autoimmunity is discussed.
Article Published Date : Apr 01, 2008
Study Type : Human: Case Report
Abstract Title:
HBV vaccine and dermatomyositis: is there an association?
Abstract Source:
Rheumatol Int. 2008 Apr;28(6):609-12. Epub 2007 Nov 23. PMID: 18034245
Article Affiliation:
Center for Autoimmune Diseases and Department of Medicine B, Sheba Tel-Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Abstract:
The etiology of dermatomyositis is unknown, but immune mechanisms play an important role. Several dermatological manifestations have been reported among carriers of hepatitis B surface antigen, and after vaccination with the HBV vaccine. Almost all the skin reactions described were peculiar skin eruptions suggestive of an immune complex reaction. Some authors described the occurrence of dermatomyositis after BCG and influenza vaccination. We report a case of a 6-year-old child, who was vaccinated for hepatitis B virus and developed a flu-like disease accompanied by a skin rash, which had the typical features of dermatomyositis. The association of vaccination with autoimmunity is discussed.
Article Published Date : Apr 01, 2008
Study Type : Human: Case Report
Clonal expansion is the process by which daughter cells arise from a parent cell. During B cell clonal expansion, many copies of that B cell are produced that share affinity with and specificity of the same antigen.
Necrosis (from the Greek νέκρωσις “death, the stage of dying, the act of killing” from νεκρός “dead”) is a form of cell injury which results in the premature death of cells in living tissue by autolysis.
[vc_row full_width=”stretch_row” content_placement=”top” woodmart_bg_position=”right-top” row_reverse_mobile=”1″ row_reverse_tablet=”1″ woodmart_disable_overflow=”1″ css=”.vc_custom_1539348987196{padding-top: 8vh !important;padding-bottom: 4vh !important;background-image: url(https://aiun.ltd/wp-content/uploads/2019/12/motorcycle-hotspot-background.jpg) !important;background-position: 0 0 !important;background-repeat: no-repeat !important;}” woodmart_parallax=”0″ woodmart_gradient_switch=”no”][vc_column css=”.vc_custom_1535720256088{margin-bottom: 4vh !important;padding-top: 0px !important;}” offset=”vc_col-lg-8 vc_col-md-7″][woodmart_image_hotspot img=”4945″ img_size=”full”][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”53.358||88.164″ title=”Tumor necrosis factor alpha or TNFα” link_text=”https://aiun.ltd/immune-system-components/tumor-necrosis-factor-alpha-or-tnf%ce%b1/”]Tumor necrosis factor (TNF, cachexin, or cachectin; once named as tumor necrosis factor alpha or TNFα) is a cell signaling protein (cytokine) involved in systemic inflammation and is one of the cytokines that make up the acute phase reaction. It is produced chiefly by activated macrophages, although it can be produced by many other cell types such as CD4+ lymphocytes, NK cells, neutrophils, mast cells, eosinophils, and neurons.[5] TNF is a member of the TNF superfamily, consisting of various transmembrane proteins with a homologous TNF domain.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”41.304||68.675″ title=”ICAM-1″ link=”url:https%3A%2F%2Faiun.ltd%2Ficam-1%2F|title:ICAM-1||”]ICAM-1 also known as CD54 is a protein that in humans is encoded by the ICAM1 gene. This gene encodes a cell surface glycoprotein which is typically expressed on endothelial cells and cells of the immune system.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”41.486||57.349″ title=”VCAM-1″ link_text=”https://aiun.ltd/vcam-1/”]Vascular cell adhesion protein 1 also known as vascular cell adhesion molecule 1 or cluster of differentiation 106 is a protein that in humans is encoded by the VCAM1 gene. VCAM-1 functions as a cell adhesion molecule[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”25.725||66.024″ title=”LFA-1″ link_text=”https://aiun.ltd/lfa-1/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”26.268||74.217″ title=”VLA-4″ link_text=”https://aiun.ltd/vla-4/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”26.087||82.410″ title=”MaC-1″ link_text=”https://aiun.ltd/mac-1/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”11.413||89.880″ title=”Chemokines” link_text=”https://aiun.ltd/chemokines/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”81.341||63.373″ title=”STAT 1″ link_text=”https://aiun.ltd/stat-1/”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”80.978||67.229″ title=”MHC-1″][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”60.688||84.337″ title=”Nitric Oxide”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”85.688||74.458″ title=”Cathespin”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”84.783||77.349″ title=”HSP 70-90″][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”82.428||84.337″ title=”NCAM”][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”86.413||91.566″ title=”MxA”]The human MxA protein is part of the antiviral state induced by alpha/beta interferon MxA inhibits the multiplication of several RNA viruses in cell[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”5.435||58.795″ title=”Cytokines”]Cytokines are a broad and loose category of small proteins that are important in cell signaling. Cytokines are peptides, and cannot cross the lipid bilayer of cells to enter the cytoplasm. Cytokines have been shown to be involved in autocrine, paracrine and endocrine signaling as immunomodulating agents.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”MAC”]The membrane-attack complex (MAC) forms transmembrane channels. These channels disrupt the cell membrane of target cells, leading to cell lysis and death.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”35.688||48.675″ title=”M1-2″]Mi-2/NuRD (Nucleosome Remodeling Deacetylase) complex, is a group of associated proteins with both ATP-dependent chromatin remodeling and histone deacetylase activities[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”C3b NEO” hotspot=”46.377||39.759″]The MAC and the immune complex-specific C3bNEO fragment are deposited on the endomysial capillaries early in the disease and lead sequentially to loss of capillaries, muscle ischemia, muscle fiber necrosis, and perifascicular atrophy.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”C3a” hotspot=”7.790||48.193″]C3a is a 77 residue anaphylatoxin that binds to the C3a receptor (C3aR), a class A G protein-coupled receptor. It plays a large role in the immune response.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” title=”TGF-b”]Transforming growth factor beta is a multifunctional cytokine belonging to the transforming growth factor superfamily that includes three different mammalian isoforms and many other signaling proteins.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”52.717||71.566″ title=”B cells”]B cells, also known as B lymphocytes, are a type of white blood cell of the small lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system by secreting antibodies[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”79.710||88.193″ title=”Mi-1″]The Mi-2/NuRD (Nucleosome Remodeling Deacetylase) complex, is a group of associated proteins with both ATP-dependent chromatin remodeling and histone deacetylase activities.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”53.442||22.892″ title=”Endothelial cell wall”]Endothelium refers to cells that line the interior surface of blood vessels and lymphatic vessels, forming an interface between circulating blood or lymph in the lumen and the rest of the vessel wall.[/woodmart_hotspot][/woodmart_image_hotspot][vc_empty_space height=”6vh” woodmart_hide_large=”0″ woodmart_hide_medium=”0″ woodmart_hide_small=”0″ woodmart_hide_extra_small=”0″][/vc_column][vc_column css=”.vc_custom_1535720261744{margin-bottom: 4vh !important;padding-top: 0px !important;}” offset=”vc_col-lg-4 vc_col-md-5 vc_col-xs-12″][woodmart_responsive_text_block size=”custom” font_weight=”600″ color_scheme=”light” align=”left” css=”.vc_custom_1539595667890{margin-bottom: 15px !important;}” woodmart_css_id=”5bc45d8c5aadf” text_font_size=”eyJwYXJhbV90eXBlIjoid29vZG1hcnRfcmVzcG9uc2l2ZV9zaXplIiwiY3NzX2FyZ3MiOnsiZm9udC1zaXplIjpbIiAud29vZG1hcnQtdGV4dC1ibG9jayJdfSwic2VsZWN0b3JfaWQiOiI1YmM0NWQ4YzVhYWRmIiwiZGF0YSI6eyJkZXNrdG9wIjoiNDVweCIsInRhYmxldCI6IjMycHgiLCJtb2JpbGUiOiIyOHB4In19″ content_width=”100″]Together
This Affords a Sporty Position.[/woodmart_responsive_text_block][woodmart_responsive_text_block font=”text” size=”custom” color_scheme=”light” align=”left” desktop_text_size=”14″ css=”.vc_custom_1536053818138{margin-bottom: 25px !important;}”]Nor again is there anyone who loves or pursues or desires to obtain pain of itself, because it is pain, but because occasionally circumstances toil.[/woodmart_responsive_text_block][woodmart_button color="primary" shape="semi-round" align="left" title="View More" link="url:%23|||"][/vc_column][/vc_row][vc_row css=”.vc_custom_1540209170412{margin-right: -10px !important;margin-left: -10px !important;}” woodmart_parallax=”0″ woodmart_gradient_switch=”no” row_reverse_mobile=”0″ row_reverse_tablet=”0″ woodmart_disable_overflow=”0″][vc_column css=”.vc_custom_1539349812585{margin-top: -8vh !important;margin-bottom: 6vh !important;padding-top: 0px !important;}” woodmart_parallax=”0″ parallax_scroll=”no” woodmart_sticky_column=”false”][vc_row_inner][vc_column_inner width=”1/2″ css=”.vc_custom_1539352179865{margin-bottom: 20px !important;padding-top: 0px !important;padding-right: 10px !important;padding-left: 10px !important;}” offset=”vc_col-lg-4 vc_col-md-4 vc_col-xs-12″][vc_video align=”center” poster_image=”3646″ image_poster_switch=”yes” img_size=”full” css=”.vc_custom_1539343713801{margin-bottom: 0px !important;}”][/vc_column_inner][vc_column_inner width=”1/2″ css=”.vc_custom_1539352192339{margin-bottom: 20px !important;padding-top: 0px !important;padding-right: 10px !important;padding-left: 10px !important;}” offset=”vc_col-lg-4 vc_col-md-4 vc_col-xs-12″][vc_video align=”center” poster_image=”3646″ image_poster_switch=”yes” img_size=”full” css=”.vc_custom_1539343722747{margin-bottom: 0px !important;}”][/vc_column_inner][vc_column_inner width=”1/3″ css=”.vc_custom_1539352202225{margin-bottom: 20px !important;padding-top: 0px !important;padding-right: 10px !important;padding-left: 10px !important;}” offset=”vc_col-lg-4 vc_col-md-4 vc_hidden-sm vc_col-xs-12″][vc_video align=”center” poster_image=”3646″ image_poster_switch=”yes” img_size=”full” css=”.vc_custom_1539343731880{margin-bottom: 0px !important;}”][/vc_column_inner][/vc_row_inner][/vc_column][/vc_row][vc_row][vc_column][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Dermatomyositis may be associated with celiac disease even in the absence of gastrointestinal symptoms.” tab_id=”1581776528917-f0335080-cdfa”][vc_column_text text_larger=”no”]
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[/vc_column_text][/vc_tta_section][/vc_tta_accordion][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Creatine supplements combined with home exercises improve functional performance without significant adverse effects in patients with polymyositis or dermatomyositis.” tab_id=”1581778867862-eabb7c70-1bc6″][vc_column_text text_larger=”no”]
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[/vc_column_text][/vc_tta_section][/vc_tta_accordion][vc_tta_accordion style=”modern” active_section=”1″ collapsible_all=”true”][vc_tta_section title=”Hepatitis B vaccine associated with dermatomyositis has been reported.” tab_id=”1581779431459-844f61df-47a9″][vc_column_text text_larger=”no”]
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[/vc_column_text][/vc_tta_section][/vc_tta_accordion][/vc_column][/vc_row][vc_row full_width=”stretch_row” content_placement=”middle” woodmart_disable_overflow=”1″ css=”.vc_custom_1535543746090{margin-bottom: 8vh !important;padding-top: 8vh !important;padding-bottom: 4vh !important;background-image: url(https://aiun.ltd/wp-content/uploads/2019/12/wood-main-bg.jpg) !important;background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}”][vc_column css=”.vc_custom_1530611754151{padding-top: 0px !important;}”][woodmart_responsive_text_block size=”custom” font_weight=”600″ color_scheme=”dark” content_width=”50″ css=”.vc_custom_1581781702522{margin-bottom: 35px !important;}” woodmart_css_id=”5e4812b24d599″ text_font_size=”eyJwYXJhbV90eXBlIjoid29vZG1hcnRfcmVzcG9uc2l2ZV9zaXplIiwiY3NzX2FyZ3MiOnsiZm9udC1zaXplIjpbIiAud29vZG1hcnQtdGV4dC1ibG9jayJdfSwic2VsZWN0b3JfaWQiOiI1ZTQ4MTJiMjRkNTk5IiwiZGF0YSI6eyJkZXNrdG9wIjoiNjRweCIsInRhYmxldCI6IjM4cHgiLCJtb2JpbGUiOiIzMnB4In19″ inline=”no”]Dermatomyositis cell pathogenesis.[/woodmart_responsive_text_block][/vc_column][vc_column width=”2/3″ offset=”vc_col-sm-offset-2″ css=”.vc_custom_1533116005031{padding-top: 0px !important;}” el_class=”z-index-100″][woodmart_image_hotspot img=”4998″ img_size=”full”][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”53.261||3.441″ title=”Antigen” link_text=”Antigen” link=”url:https%3A%2F%2Faiun.ltd%2Fantigen%2F|title:Antigen|target:%20_blank|”]A toxin or other foreign substance which induces an immune response in the body, especially the production of antibodies.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”88.225||22.267″ title=”Clonal expansion” link_text=”Clonal expansion” link=”url:https%3A%2F%2Faiun.ltd%2Fclonal-expansion%2F|title:Clonal%20expansion%C2%A0||”]
[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”right” hotspot=”48.370||17.611″ title=”MCH” link_text=”MCH” link=”url:https%3A%2F%2Faiun.ltd%2Fmajor-histocompatibility-complex-mhc%2F|||”]The MHC molecules are glycoproteins encoded in a large cluster of genes located on chromosome 6. They were first identified by their potent effect on the immune response to transplanted tissue
The principal function of the MHC is to present antigen to T cells to discriminate between self (our cells and tissues) and nonself (the invaders or modified self).[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot_dropdown_side=”top” hotspot=”10.145||65.789″ title=”T-cell receptor (TCR)” link_text=”T-cell receptor (TCR)” link=”url:https%3A%2F%2Faiun.ltd%2Ft-cell-receptor-tcr%2F|title:T-cell%20receptor%C2%A0(TCR)||”]The T-cell receptor (TCR) is a molecule found on the surface of T cells, or T lymphocytes, that is responsible for recognizing fragments of antigen as peptides bound to major histocompatibility complex (MHC) molecules.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”22.464||61.943″ title=”CD28 (Cluster of Differentiation 28)” link_text=”CD28″ link=”url:https%3A%2F%2Faiun.ltd%2Fcd28-cluster-of-differentiation-28%2F|title:CD28|target:%20_blank|”]CD28 (Cluster of Differentiation 28) is one of the proteins expressed on T cells that provide co-stimulatory signals required for T cell activation and survival. T cell stimulation through CD28 in addition to the T-cell receptor (TCR) can provide a potent signal for the production of various interleukins (IL-6 in particular).[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”34.420||61.943″ title=”CTLA-4″ link_text=”CTLA-4″ link=”url:https%3A%2F%2Faiun.ltd%2Fctla-4%2F|title:CTLA-4%C2%A0||”]CTLA4 or CTLA-4 (cytotoxic T-lymphocyte-associated protein 4), also known as CD152 (cluster of differentiation 152), is a protein receptor that functions as an immune checkpoint and downregulates immune responses.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”50.725||47.166″ title=”CD8″ link_text=”CD8″ link=”url:https%3A%2F%2Faiun.ltd%2Fcd8%2F|title:CD8|target:%20_blank|”]CD8 (cluster of differentiation 8) is a transmembrane glycoprotein that serves as a co-receptor for the T cell receptor (TCR).[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”34.420||38.866″ title=”Integrins” link_text=”Integrins” link=”url:https%3A%2F%2Faiun.ltd%2Fintegrins%2F|title:Integrins%C2%A0|target:%20_blank|”]Integrins are transmembrane receptors that facilitate cell-extracellular matrix (ECM) adhesion. Upon ligand binding, integrins activate signal transduction pathways that mediate cellular signals such as regulation of the cell cycle, organization of the intracellular cytoskeleton, and movement of new receptors to the cell membrane. The presence of integrins allows rapid and flexible responses to events at the cell surface (e.g. signal platelets to initiate an interaction with coagulation factors).
Several types of integrins exist, and one cell may have multiple different types on its surface. Integrins are found in all animals while integrin-like receptors are found in plant cells.
Integrins work alongside other proteins such as cadherins, the immunoglobulin superfamily cell adhesion molecules, selectins and syndecans, to mediate cell–cell and cell–matrix interaction. Ligands for integrins include fibronectin, vitronectin, collagen and laminin.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”83.877||45.142″ title=”MMPs” link_text=”MMPs” link=”url:https%3A%2F%2Faiun.ltd%2Fmatrix-metalloproteinases%2F|title:MMPs%C2%A0|target:%20_blank|”]Matrix metalloproteinases (MMPs), the main extracellular matrix (ECM) enzymes in collagen degradation, as a target for anticancer drugs.
The MMPs play an important role in tissue remodeling associated with various physiological or pathological processes such as morphogenesis, angiogenesis, tissue repair, cirrhosis, arthritis, and metastasis.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”51.630||58.502″ title=”LFA-1″ link_text=”LFA-1″ link=”url:https%3A%2F%2Faiun.ltd%2Flymphocyte-function-associated-antigen-1-lfa-1%2F|title:LFA-1|target:%20_blank|”]Lymphocyte function-associated antigen 1 (LFA-1) is an integrin found on lymphocytes and other leukocytes.[1] LFA-1 plays a key role in emigration, which is the process by which leukocytes leave the bloodstream to enter the tissues. LFA-1 also mediates firm arrest of leukocytes.[2] Additionally, LFA-1 is involved in the process of cytotoxic T cell mediated killing as well as antibody mediated killing by granulocytes and monocytes.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”65.036||59.109″ title=”MMP-9″ link_text=”MMP-9″ link=”url:https%3A%2F%2Faiun.ltd%2Fmmp-9%2F|title:MMP-9|target:%20_blank|”]Matrix metallopeptidase 9 (MMP-9), also known as 92 kDa type IV collagenase, 92 kDa gelatinase or gelatinase B (GELB), is a matrixin, a class of enzymes that belong to the zinc-metalloproteinases family involved in the degradation of the extracellular matrix.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”13.587||7.692″ title=”Macrophage” link_text=”Macrophage” link=”url:https%3A%2F%2Faiun.ltd%2Fmacrophage%2F|title:macrophage|target:%20_blank|”]A macrophage is a type of phagocyte, which is a cell responsible for detecting, engulfing and destroying pathogens and apoptotic cells. Macrophages are produced through the differentiation of monocytes, which turn into macrophages when they leave the blood.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”60.507||53.846″ title=”IFNγ” link_text=”IFNγ” link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5033%26preview%3Dtrue|title:IFN%CE%B3|target:%20_blank|”]Interferon gamma (IFNγ) is a dimerized soluble cytokine that is the only member of the type II class of interferons. The existence of this interferon, which early in its history was known as immune interferon, was described by E. F. Wheelock as a product of human leukocytes stimulated with phytohemagglutinin, and by others as a product of antigen-stimulated lymphocytes.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”80.072||57.895″ title=”IL-2″ link_text=”IL-2″ link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5034%26preview%3Dtrue|title:IL-2|target:%20_blank|”]Interleukin-2 (IL-2) is an interleukin, a type of cytokine signaling molecule in the immune system. It is a 15.5 – 16 kDa protein that regulates the activities of white blood cells (leukocytes, often lymphocytes) that are responsible for immunity. IL-2 is part of the body’s natural response to microbial infection, and in discriminating between foreign (“non-self”) and “self”. IL-2 mediates its effects by binding to IL-2 receptors, which are expressed by lymphocytes. The major sources of IL-2 are activated CD4+ T cells and activated CD8+ T cells.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”63.225||73.684″ title=”Perforin” link_text=”Perforin” link=”url:https%3A%2F%2Faiun.ltd%2Fperforin%2F|title:Perforin|target:%20_blank|”]Perforin is a pore forming cytolytic protein found in the granules of cytotoxic T lymphocytes (CTLs) and natural killer cells (NK cells). Upon degranulation, perforin binds to the target cell’s plasma membrane, and oligomerises in a Ca2+ dependent manner to form pores on the target cell.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”29.529||41.903″ title=”Chemokines” link_text=”Chemokines” link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5038%26preview%3Dtrue|title:Chemokines%C2%A0|target:%20_blank|”]Chemokines (Greek -kinos, movement) are a family of small cytokines, or signaling proteins secreted by cells. Their name is derived from their ability to induce directed chemotaxis in nearby responsive cells; they are chemotactic cytokines.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”87.862||87.045″ title=”Necrosis” link_text=”Necrosis” link=”url:https%3A%2F%2Faiun.ltd%2Fnecrosis%2F|title:Necrosis%C2%A0|target:%20_blank|”]
[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”33.877||70.445″ title=”BB1″ link_text=”BB1 monoclonal antibodies”]BB1 monoclonal antibodies[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”57.246||72.672″ title=”MMP-2″ link_text=”MMP-2″ link=”url:https%3A%2F%2Faiun.ltd%2Fmmp-2%2F|title:MMP-2|target:%20_blank|”]72 kDa type IV collagenase also known as matrix metalloproteinase-2 (MMP-2) and gelatinase A is an enzyme that in humans is encoded by the MMP2 gene.[5] The MMP2 gene is located on chromosome 16 at position 12.2[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”42.572||94.130″ img_size=”TAP” link_text=”TAP” link=”url:https%3A%2F%2Faiun.ltd%2F%3Fpage_id%3D5049%26preview%3Dtrue|title:TAP|target:%20_blank|”]Transporter associated with antigen processing (TAP) protein complex belongs to the ATP-binding-cassette transporter family.[1] It delivers cytosolic peptides into the endoplasmic reticulum (ER), where they bind to nascent MHC class I molecules[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”31.703||97.773″ title=”ER” link_text=”ER” link=”url:https%3A%2F%2Faiun.ltd%2Fthe-endoplasmic-reticulum-er%2F|title:ER|target:%20_blank|”]The endoplasmic reticulum (ER) is a type of organelle made up of two subunits – rough endoplasmic reticulum (RER), and smooth endoplasmic reticulum (SER). The endoplasmic reticulum is found in most eukaryotic cells and forms an interconnected network of flattened, membrane-enclosed sacs known as cisternae (in the RER), and tubular structures in the SER. The membranes of the ER are continuous with the outer nuclear membrane. The endoplasmic reticulum is not found in red blood cells, or spermatozoa.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”56.341||77.126″ title=”Calnexin” link_text=”Calnexin” link=”url:https%3A%2F%2Faiun.ltd%2Fcalnexin%2F|title:Calnexin|target:%20_blank|”]Calnexin is a chaperone, characterized by assisting protein folding and quality control, ensuring that only properly folded and assembled proteins proceed further along the secretory pathway. It specifically acts to retain unfolded or unassembled N-linked glycoproteins in the ER.[/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”73.188||60.931″ title=”IL-1α” link_text=”IL-1α” link=”url:https%3A%2F%2Faiun.ltd%2Finterleukin-1-alpha-il-1%25ce%25b1%2F|title:IL-1%CE%B1|target:%20_blank|”]Interleukin 1 alpha (IL-1α) also known as hematopoietin 1 is a cytokine of the interleukin 1 family that in humans is encoded by the IL1A gene. In general, Interleukin 1 is responsible for the production of inflammation, as well as the promotion of fever and sepsis. IL-1α inhibitors are being developed to interrupt those processes and treat diseases.[/woodmart_hotspot][woodmart_hotspot][/woodmart_hotspot][woodmart_hotspot hotspot_type=”text” hotspot=”22.464||47.773″ title=”MIG” link_text=”MIG” link=”url:https%3A%2F%2Faiun.ltd%2Fmig%2F|title:MIG|target:%20_blank|”]MIG, a member of the CXC subfamily of chemokines is an inflammatory chemokine that is important in the recruitment of activated T-cells to sites of infection. MIG enhances Th1 and Th2 polarization, attracting Th1 cells and inhibiting Th2 migration.[/woodmart_hotspot][/woodmart_image_hotspot][/vc_column][/vc_row][vc_row full_width=”stretch_row” content_placement=”middle” woodmart_disable_overflow=”1″ css=”.vc_custom_1535543746090{margin-bottom: 8vh !important;padding-top: 8vh !important;padding-bottom: 4vh !important;background-image: url(https://aiun.ltd/wp-content/uploads/2019/12/wood-main-bg.jpg) !important;background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}”][vc_column css=”.vc_custom_1530611754151{padding-top: 0px !important;}”][woodmart_responsive_text_block size=”custom” font_weight=”600″ color_scheme=”dark” content_width=”50″ desktop_text_size=”64″ tablet_text_size=”38″ mobile_text_size=”32″ css=”.vc_custom_1530782250887{margin-bottom: 35px !important;}”]The Best Choice For A Winter Ride.[/woodmart_responsive_text_block][/vc_column][vc_column width=”2/3″ offset=”vc_col-sm-offset-2″ css=”.vc_custom_1533116005031{padding-top: 0px !important;}” el_class=”z-index-100″][woodmart_image_hotspot img=”5853″ woodmart_color_scheme=”light” img_size=”full”][woodmart_hotspot hotspot_dropdown_side=”right” hotspot=”85.413||37.684″ product_id=”53″][/woodmart_hotspot][woodmart_hotspot hotspot_dropdown_side=”right” hotspot=”58.509||6.985″ product_id=”53″][/woodmart_hotspot][woodmart_hotspot hotspot_dropdown_side=”right” hotspot=”33.874||34.926″ product_id=”53″][/woodmart_hotspot][woodmart_hotspot hotspot_dropdown_side=”top” hotspot=”11.507||62.684″ product_id=”53″][/woodmart_hotspot][/woodmart_image_hotspot][/vc_column][/vc_row]
Abstract Title:
Dermatomyositis associated with celiac disease: response to a gluten-free diet.
Abstract Source:
Can J Gastroenterol. 2006 Jun;20(6):433-5. PMID: 16779462
Abstract:
The association between dermatomyositis and celiac disease in children has been well documented. In the adult population, however, the association has not been clearly established. A rare case of concomitant dermatomyositis and celiac disease in a 40-year-old woman is presented. After having been diagnosed with dermatomyositis and iron deficiency anemia, this patient was referred to the gastroenterology clinic to exclude a gastrointestinal malignancy. Blood tests revealed various vitamin deficiencies consistent with malabsorption. The results of gastroscopy with duodenal biopsy were consistent with celiac disease. After she was put on a strict gluten-free diet, both nutritional deficiencies and the dermatomyositis resolved. The patient’s human leukocyte antigen haplotype study was positive for DR3 and DQ2, which have been shown to be associated with both juvenile dermatomyositis and celiac disease. It is suggested that patients with newly diagnosed dermatomyositis be investigated for concomitant celiac disease even in the absence of gastrointestinal symptoms.
Article Published Date : Jun 01, 2006
Study Type : Human Study
Abstract Title:
Lupus erythematosus and other autoimmune diseases related to statin therapy: a systematic review.
Abstract Source:
J Eur Acad Dermatol Venereol. 2007 Jan ;21(1):17-24. PMID: 17207162
Article Affiliation:
Department of Dermatology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. bernard.noel@chuv.hospvd.ch
Abstract:
BACKGROUND: Statins have been increasingly associated with drug-induced autoimmune reactions, including lupus erythematosus.
OBJECTIVE: To identify and determine the clinical and biological characteristics of statin-induced autoimmune reactions.
MATERIAL AND METHODS: The MEDLINE database (1966 to September 2005) was used to identify all reported cases of statin-induced autoimmune diseases. The keywords used were statins, 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors, adverse effects, autoimmune disease, lupus erythematosus, dermatomyositis and polymyositis.
RESULTS: Twenty-eight cases of statin-induced autoimmune diseases have been published so far. Systemic lupus erythematosus was reported in 10 cases, subacute cutaneous lupus erythematosus in three cases, dermatomyositis and polymyositis in 14 cases and lichen planus pemphigoides in one case. Autoimmune hepatitis was observed in two patients with systemic lupus erythematosus. The mean time of exposure before disease onset was 12.8+/-18 months; range 1 month-6 years. Systemic immunosuppressive therapy was required in the majority of cases. In many patients, antinuclear antibodies were still positive many months after clinical recovery. A lethal outcome has been recorded in two patients despite aggressive immunosuppressive therapy.
CONCLUSION: Long-term exposure to statins may be associated with drug-induced lupus erythematosus and other autoimmune disorders. Fatal cases have been reported despite early drug discontinuation and aggressive systemic immunosuppressive therapy.
Article Published Date : Jan 01, 2007
Study Type : Human Study
PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
About this article
Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
- Published
- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Abstract Title:
The effect of cigarette smoking on the clinical and serological phenotypes of polymyositis and dermatomyositis.
Abstract Source:
Semin Arthritis Rheum. 2018 Feb 14. Epub 2018 Feb 14. PMID: 29703532
Article Affiliation:
Adam Schiffenbauer
Abstract:
OBJECTIVE: Cigarette smoking is associated with immune-mediated disorders. We explored the contribution of smoking to polymyositis (PM) and dermatomyositis (DM) phenotypes and attempted to determine whether cigarette smoking effects differ by race and genotype.
METHODS: Associations of tobacco smoking with disease features, autoantibodies, HLA types, and race were evaluated using multiple logistic regressions in 465 patients.
RESULTS: Caucasian ever-smokers (n = 140) were more likely to have PM (adjusted OR = 2.24, 95% CI: 1.41-3.57), anti-synthetase (adjusted OR = 1.93, 95% CI: 1.12-3.34) and anti-Jo-1 autoantibodies (adjusted OR = 1.94, 95% CI: 1.08-3.46) and less likely to have anti-p155/140 autoantibodies (adjusted OR = 0.36, 95% CI: 0.14-0.92). In Caucasians, ever-smokers had a greater interstitial lung disease (ILD) frequency than never-smokers, while in African-Americans this relationship was inverted, but neither trend reached statistical significance. Pack-years of cigarette smoking showed significant positive associations with PM (adjusted OR = 1.02, 95% CI: 1.002-1.04) and ILD (adjusted OR = 1.02, 95% CI: 1.001-1.03) and was inversely associated with anti-p155/140 autoantibodies (adjusted OR = 0.93, 95% CI: 0.87-0.99) in Caucasians. Caucasian heavy smokers (≥20 pack-years) were more likely to have PM (adjusted OR = 2.52, 95% CI: 1.25-5.09), ILD (adjusted OR = 2.48, 95% CI: 1.23-5.00) and anti-Jo-1 autoantibodies (adjusted OR = 2.65, 95% CI: 1.16-6.08) than never-smokers. In Caucasians, compared to never-smokers without HLA-DRB1*03:01 allele, ever-smokers with HLA-DRB1*03:01 allele had the highest odds of PM, ILD, ASA, and anti-Jo-1 autoantibodies. Risks for those with only one of these two factors were intermediate. An inverse pattern was observed regarding anti-p155/140 autoantibodies.
CONCLUSION: Tobacco smoking was associated with clinical and autoantibody phenotypes in Caucasians. Our findings also suggest possible interactions among HLA-DRB1*03:01 and smoking on the risk of PM and ILD, as well as, anti-synthetase, anti-Jo-1, and anti-p155/140 autoantibodies in Caucasians.
Article Published Date : Feb 13, 2018
Study Type : Human Study
Abstract Title:
Dermatomyositis-like syndrome and HMG-CoA reductase inhibitor (statin) intake.
Abstract Source:
Muscle Nerve. 2004 Dec ;30(6):803-7. PMID: 15389654
Article Affiliation:
Department of Neurology, Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA.
Abstract:
A patient developed an adult-onset dermatomyositis-like syndrome characterized by skin rash and progressive proximal muscle weakness concurrent with the intake of simvastatin. Despite discontinuation of the statin, symptoms progressed and required conventional steroid therapy for remission. The association between statins and the development of a musculocutaneous syndrome closely resembling dermatomyositis in susceptible subjects is poorly understood and has been reported rarely. The purpose of this report is to provide additional support for this pathological association. Since the population receiving statins is large and rapidly growing, caregivers are urged to be alert regarding the early recognition and proper care of the spectrum of neuromuscular complications linked to statin intake.
Article Published Date : Dec 01, 2004
Study Type : Human: Case Report
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
About this article
Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
- Published
- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Creatine supplements in patients with idiopathic inflammatory myopathies who are clinically weak after conventional pharmacologic treatment: Six-month, double-blind, randomized, placebo-controlled trial
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
Rights and permissions
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
About this article
Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
- Published
- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Abstract Title:
[Fluvastatin-induced dermatomyositis].
Abstract Source:
Ann Dermatol Venereol. 2005 Dec ;132(12 Pt 1):996-9. PMID: 16446645
Article Affiliation:
Service de Dermatologie, Centre Hospitalier Universitaire de Caen.
Abstract:
BACKGROUND: Dermatomyositis is a rare inflammatory dermatosis for which an iatrogenic origin has been described in very few cases. We report a case of dermatomyositis occurring after fluvastatin intake.
CASE REPORT: A 76-year-old male patient sought medical attention for a photodistributed rash and considerable muscular weakness present for one month. Two months earlier, fluvastatin had been introduced following the discovery of dyslipidemia. Serum creatine phosphokinase levels were elevated. Histological examination of a muscle biopsy was consistent with a diagnosis of dermatomyositis. Investigation for neoplasia and associated autoimmune disease proved negative. All clinical and laboratory abnormalities diminished spontaneously without recourse to corticosteroids within one month of the final intake of fluvastatin. After a follow-up period of three years, no recurrence was observed and no signs of neoplasia or connectivitis were found.
DISCUSSION: Iatrogenic dermatomyositis has only been reported in rare cases. Certain drugs have been incriminated, notably D-penicillamine. Six cases of drug-induced dermatomyositis have been described with statins: simvastatin, atorvastatin, pravastatin and lovastatin. Of these cases, only one resolved spontaneously after withdrawal of the drug alone without use of corticosteroids. Our case concerns intake of fluvastatin, an HMG-CoA reductase inhibitor with rare though well-known muscular side effects: elevated serum CPK, myalgia and rhabdomyolysis. Six cases of polymyositis have also been reported. Ours is the first case of dermatomyositis described with this category of statins. It is also the second reported case showing improvement after withdrawal of the lipid-lowering agent and without corticosteroids. Thus in cases of dermatomyositis, this iatrogenic picture should be sought routinely.
Article Published Date : Dec 01, 2005
Study Type : Human: Case Report
Pediatric Rheumatology volume 11, Article number: P26 (2013)
Introduction
Juvenile Dermatomyositis (JDM) is an inflammatory myopathy with a predilection for proximal muscles and skin. Current treatment of JDM includes early aggressive use of corticosteroids coupled with immunosuppressive drugs such as methotrexate, cyclosporine and intravenous immune globulins (IVIG). Delay to diagnosis and inadequate treatment increase the risk of developing calcinosis. However, once estabilished, this complication is difficult to treat.
Objectives
We describe a case of JDM with severe skin manifestations including ulcerations and diffuse calcinosis, poorly responsive to conventional therapy.
Methods
Case report. A caucasian boy presented at the age of 3 years and 10 months with malaise, fatigue, arthralgia, heliotrope rash, Gottron’s papules on PIP joints, elbows and knees, shawl sign, and periungueal telangectasia. There was weakness of proximal muscles, elevation of aldolase (11.0 U/L, normal < 7.3 U/L), normal serum lactate dehydrogenase (LDH) and creatine kinase (CK), and negative anti-nuclear antibody. Magnetic resonance imaging (MRI) demonstrated diffuse muscle edema of proximal muscles and electromyography showed a myopathic pattern. Despite treatment with steroids pulses, followed by daily oral steroids and hydroxycloroquine, his illness was complicated by skin ulcerations of the upper and lower extremities and widespread calcinosis. X-ray of extremities showed superficial diffuse nodules and plaques. The patient still had elevation of aldolase (19.3 U/L) and a Childhood Myositis Assessment Scale (CMAS) score of 35 out of 52, with a Manual Muscle Testing (MMT8) score of 50 out 80. So a treatment with Methotrexate was added, but his areas of calcinosis and ulcerations continued to spread, so alendronate was introduced (two years from disease onset), but without benefit. On the basis of a published report (Arabshahi B, et al. J Pediatr 2012) treatment with topical sodium thiosulfate was initiated. Thiosulphate was used initially at 3% concentration, and subsequently increased to 10% concentration, applied to calcification of upper and lower extremities daily under occlusive dressing.
Results
After 9 months of therapy, a significant improvement of calcinosis and ulcerations was noted, together with lack of progression. Photos, with the family consent, were taken before and after treatment.
Conclusion
Treatment of calcinosis in JDM is very difficult. Several agents have been used, such as calcium channel blockers, probenecid, colchicine, tumor necrosis factors inhibitors, bisphosphonates, and intra-lesional corticosteroids. None of these however has shown to be consistently effective and no controlled trial exist. Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Topical use has been described for ulcerations associated with lupus calcinosis and uremic calciphylaxis. However, the use of this agent for treatment of calcinosis associated to dermatomyositis has been reported only once in literature.
We hypothesize that sodium thiosulfate may have a role in stabilizing or improving calcinosis, and in diminishing pain and promoting revascularization of cutaneous ulcerations. A controlled study in order to determinate the safety and efficacy of this treatment in JDM is currently planned.
Disclosure of interest
None declared.
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Cite this article
Pagnini, I., Simonini, G., Giani, T. et al. PReS-FINAL-2013: Sodium thiosulfate for the treatment of Juvenile Dermatomyositis complicated by calcinosis. Pediatr Rheumatol 11, P26 (2013). https://doi.org/10.1186/1546-0096-11-S2-P26
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- DOIhttps://doi.org/10.1186/1546-0096-11-S2-P26
Abstract Title:
HBV vaccine and dermatomyositis: is there an association?
Abstract Source:
Rheumatol Int. 2008 Apr;28(6):609-12. Epub 2007 Nov 23. PMID: 18034245
Article Affiliation:
Center for Autoimmune Diseases and Department of Medicine B, Sheba Tel-Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Abstract:
The etiology of dermatomyositis is unknown, but immune mechanisms play an important role. Several dermatological manifestations have been reported among carriers of hepatitis B surface antigen, and after vaccination with the HBV vaccine. Almost all the skin reactions described were peculiar skin eruptions suggestive of an immune complex reaction. Some authors described the occurrence of dermatomyositis after BCG and influenza vaccination. We report a case of a 6-year-old child, who was vaccinated for hepatitis B virus and developed a flu-like disease accompanied by a skin rash, which had the typical features of dermatomyositis. The association of vaccination with autoimmunity is discussed.
Article Published Date : Apr 01, 2008
Study Type : Human: Case Report
Abstract Title:
HBV vaccine and dermatomyositis: is there an association?
Abstract Source:
Rheumatol Int. 2008 Apr;28(6):609-12. Epub 2007 Nov 23. PMID: 18034245
Article Affiliation:
Center for Autoimmune Diseases and Department of Medicine B, Sheba Tel-Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Abstract:
The etiology of dermatomyositis is unknown, but immune mechanisms play an important role. Several dermatological manifestations have been reported among carriers of hepatitis B surface antigen, and after vaccination with the HBV vaccine. Almost all the skin reactions described were peculiar skin eruptions suggestive of an immune complex reaction. Some authors described the occurrence of dermatomyositis after BCG and influenza vaccination. We report a case of a 6-year-old child, who was vaccinated for hepatitis B virus and developed a flu-like disease accompanied by a skin rash, which had the typical features of dermatomyositis. The association of vaccination with autoimmunity is discussed.
Article Published Date : Apr 01, 2008
Study Type : Human: Case Report
Clonal expansion is the process by which daughter cells arise from a parent cell. During B cell clonal expansion, many copies of that B cell are produced that share affinity with and specificity of the same antigen.
Necrosis (from the Greek νέκρωσις “death, the stage of dying, the act of killing” from νεκρός “dead”) is a form of cell injury which results in the premature death of cells in living tissue by autolysis.