Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Von Willebrand factor antigen (vWF: Ag) is known to be produced and excreted by endothelial cells (EC). The influences of interferon (IFN) on surface, excreted and intracellular vWF: Ag amounts of EC were studied by flow cytometry and ELISA. Serum levels of vWF: Ag in patients with connective tissue diseases were also studied by ELISA. Experiments in vitro showed that IFN increased vWF: Ag amounts of EC. Serum levels of vWF: Ag in patients with mixed connective tissue disease (MCTD), systemic lupus erythematosus, progressive systemic sclerosis, polymyositis/dermatomyositis or rheumatoid arthritis (RA) were significantly higher than those of normal subjects, on the other hand, vWF: Ag levels in patients with aortitis syndrome were within normal range. MCTD patients complicated with pulmonary hypertension (PH) [MCTD (PH)] and RA vasculitis (MRA) patients had quite high levels of vWF: Ag. The levels of vWF: Ag seemed to correlate with severity of PH in patients with MCTD (PH). Increased serum levels of vWF: Ag observed in those patients might be induced by EC damaged or influenced by IFN or other cytokines. Monitoring of vWF: Ag levels could be useful to predict the onset and pathologic conditions of MCTD (PH) or related vascular diseases.
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PMID:[Studies on von Willebrand factor antigen in endothelial cells and sera of patients with connective tissue diseases]. 169 33

Major histocompatibility complex class I (MHC-I) expression on target cells is a prerequisite for antigen-specific T cell-mediated cytotoxicity (TCMC). Enhanced MHC-I expression has been attributed to interferons (IFNs) released from inflammatory cells. In previous studies, we found evidence of TCMC (invasion of non-necrotic muscle fibers by cytotoxic T cells) in polymyositis (PM) and in inclusion body myositis (IBM). We occasionally found evidence of TCMC in Duchenne dystrophy (DD) but not in dermatomyositis (DM). This study examines the relationships between TCMC, MHC-I expression, and IFN immunoreactivity in these diseases and normal controls. In controls, reactivity for MHC-I was confined to blood vessels. In all diseases, regenerating fibers expressed MHC-I. In IBM, PM and DD, all nonnecrotic muscle fibers invaded by CD8+ cells and some adjacent fibers expressed MHC-I. In DM, myriad muscle fibers expressed MHC-I but none were invaded by CD8+ cells. In all diseases, only a few mononuclear cells and no muscle fiber surfaces were immunoreactive for IFNs. We conclude that MHC-I expression on muscle fibers is necessary but not sufficient for TCMC in myopathy; that the biological significance of increased MHC-I expression in DM remains undefined; and that currently available and appropriately controlled immunocytochemical methods show no relationship between increased MHC-I expression on muscle fibers and local IFN synthesis by mononuclear cells.
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PMID:Major histocompatibility complex class I antigen expression, immunolocalization of interferon subtypes, and T cell-mediated cytotoxicity in myopathies. 247 Jun 63

Tubuloreticular structures (TRS) and cylindric confronting cisternae (CCC) have been observed in circulating lymphocytes and in the muscle of six children with dermatomyositis. The presence of TRS was seen in all cases investigated, the number of CCC increased in various cells with the severity of the disease. Extensive formation of TRS and CCC in childhood dermatomyositis probably reflects local or systemic alpha-interferon production and suggests that some viral factor is responsible for the disease.
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PMID:Tubuloreticular structures (TRS) and cylindric confronting cisternae (CCC) in childhood dermatomyositis. 260 38

A significant proportion of patients relapse after allogeneic BMT for CML. These relapses have been treated by induction of a graft-versus-leukemia effect by transfusing donor leukocytes. We have treated a 27-year-old woman with interferon and donor leukocyte transfusion and a complete haematological and cytogenetic remission was obtained coincident with the onset of GVHD. Her course was complicated by prolonged and profound pancytopenia which was fully reversed by the administration of rGM-CSF. She remains in CR with mild dermatomyositis due to chronic GVHD 17 months after the procedure.
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PMID:Reinduction of remission of chronic myeloid leukemia by donor leukocyte transfusion following relapse after bone marrow transplantation: recovery complicated by initial pancytopenia and late dermatomyositis. 827 41

We recently observed a patient with chronic C hepatitis who developed first autoimmune thrombocytopenic purpura and subsequently dermatomyositis. While the association could be coincidental, it is possible that hepatitis C virus could have induced autoantibodies or circulating immune complexes which contributed to both. Although concerns are sometimes raised about the use of corticosteroids and immunosuppressive therapy to control symptoms in hepatitis C virus infected patients with rheumatic or autoimmune diseases, corticosteroid and immunosuppressive therapies are considered appropriate in cases of chronic C hepatitis with rapidly progressive autoimmune diseases. Our case illustrates the paradox that, despite the continuing presence of a viremic form of chronic C hepatitis, clinical symptoms improved with combined immunosuppressive therapy, without deterioration of the hepatitis. Our results could be the consequence of the association of immunosuppressive therapy intensive enough to control the autoimmune thrombocytopenic purpura and dermatomyositis with alpha-interferon whose antiviral capacity may have been able to prevent re-exacerbation of the hepatitis.
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PMID:Autoimmune thrombocytopenic purpura and dermatomyositis associated with chronic hepatitis C. A therapeutic dilemma. 1034 7

An association between auto-immune disorders and interferon (IFN) has been reported. High levels of natural IFNalpha are present in the blood of patients with auto-immune disease and correlate with disease activity. In addition, IFNalpha treatment of humans has resulted in multiple reports of associated auto-immune phenomena. We describe a patient who underwent resection of regionally metastatic melanoma, was given adjuvant high-dose IFNalpha2b, and subsequently developed dermatomyositis. To the authors' knowledge this is the first report of dermatomyositis in association with IFNalpha treatment. We review the literature reporting associations between IFNalpha and auto-immune disease and discuss possible mechanisms by which IFNalpha may contribute to the development of auto-immune disease. High dose IFNalpha2b is more commonly prescribed since it was approved as an adjuvant treatment for patients with surgically resected high-risk melanoma. The potential for cases of IFN-associated auto-immune disease is therefore a clinical concern. Standard side effects of high-dose IFN therapy resemble symptoms of auto-immune diseases, which may make prompt diagnosis difficult. Therefore, it is important that auto-immune diseases such as dermatomyositis are recognized as potential side effects of treatment with high-dose IFNalpha.
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PMID:Dermatomyositis after interferon alpha treatment. 1071 63

Recent advances in the study of global patterns of gene expression with the use of microarray technology, coupled with data analysis using sophisticated statistical algorithms, have provided new insights into pathogenic mechanisms of disease. Complementary and reproducible data from multiple laboratories have documented the feasibility of analysis of heterogeneous populations of peripheral blood mononuclear cells from patients with rheumatic diseases through use of this powerful technology. Although some patterns of gene expression, including increased expression of immune system cell surface activation molecules, confirm previous data obtained with other techniques, some novel genes that are differentially expressed have been identified. Most interesting is the dominant pattern of interferon-induced gene expression detected among blood mononuclear cells from patients with systemic lupus erythematosus and juvenile dermatomyositis. These data are consistent with longstanding observations indicating increased circulating interferon-alpha in the blood of patients with active lupus, but draw attention to the dominance of the interferon pathway in the hierarchy of gene expression pathways implicated in systemic autoimmunity.
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PMID:Microarray analysis of gene expression in lupus. 1468 May 3

Here we clarified the morphology and phenotype of interleukin (IL)-17- and interferon (IFN)-gamma-producing cells in both in vitro and in vivo situations. Oligoclonal activation of normal peripheral blood mononuclear cells with the superantigen Staphylococcus aureus enterotoxin B and polyclonal activation with phorbol myristate acetate/phytohemagglutinin were used as in vitro models. This study was extended to various in vivo situations such as rheumatoid arthritis, dermatomyositis, and normal activated lymph nodes. The phenotype of IL-17- and IFN-gamma-producing cells was evaluated by immunohistochemistry using the CD3 and CD4 T-cell markers, the CD20, CD38, kappa and lambda light chain B-cell lineage markers. The expression of two chemokine receptors, CCR6 and CCR7, involved with their associated ligands CCL20 and CCL19/CCL21 in the migration of T lymphocytes, was evaluated in tissue sections. After both polyclonal and oligoclonal activation, IL-17+ and IFN-gamma+ cells acquired a plasma cell-like morphology associated with a high secretory activity, the reduced expression of CD3, and no change of CD4 expression. In rheumatoid arthritis, dermatomyositis, and activated lymph nodes, both IL-17- and IFN-gamma-producing cells had the same morphology. These Th1 cytokine-producing cells were CD4(+)-, CD3-, and B-cell lineage marker-negative. In both in vitro and in vivo situations, expression of CCR6 or CCR7 was not associated with a particular subset. In conclusion, activated T-helper CD4(+) T cells, by their release of cytokines, seem to have functional similarities with plasma cells secreting immunoglobulins.
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PMID:Plasma cell-like morphology of Th1-cytokine-producing cells associated with the loss of CD3 expression. 1474 47

Mixed cryoglobulinaemia (MC) is a systemic vasculitis involving small vessels (arterioles, capillaries, venules). The histological hallmark of the disease is the leukocytoclastic vasculitis secondary to the vascular deposition of circulating immune-complexes (CIC), mainly cryoglobulins and complement. The immune-mediated vasculitic lesions are responsible for different MC clinical features, including cutaneous and visceral organ involvement. Hepatitis C virus (HCV) represents the triggering factor in the large majority of MC patients (>90%). Moreover, several epidemiological, clinico-pathological and laboratory investigations suggested a possible role for HCV in a wide spectrum of immuno-lymphoproliferative disorders; namely, porphyria cutanea tarda, diabetes, polyarthritis, lung fibrosis, poly-dermatomyositis, thyroiditis, thyroid cancer, B-cell non-Hodgkin's lymphomas (B-NHL), etc. Renal involvement with or without MC syndrome can be observed in HCV-infected individuals. There is great geographical etherogeneity in the prevalence of HCV-related disorders. This epidemiological observation suggests a multifactorial and multistep process in the pathogenesis of these conditions, involving other unknown genetic and/or environmental factors. HCV lymphotropism may explain the mono-oligoclonal B-lymphocyte expansion observed in HCV-infected individuals, particularly in MC patients. The 'benign' lymphoproliferative disorder, classified as monotypic lymphoproliferative disorders of undetermined significance (MLDUS), may be responsible for the wide production of CIC, including cryoglobulins, rheumatoid factor and different organ and non-organ specific autoantibodies. The consequence is the appearance of various HCV-related autoimmune diseases, including MC syndrome. This latter may be complicated by B-NHL in 10% of the cases; moreover, HCV infection has been confirmed in a significant percentage of 'idiopathic B-NHL. For a correct therapeutic approach to cryoglobulinaemic vasculitis, as well as to other HCV-related disorders, we should deal with concomitant, conflicting conditions: HCV infection, autoimmune and lymphoproliferative alterations. In this scenario, we can treat the diseases at three different levels by means of etiologic, pathogenetic and/or symptomatic therapies. The eradication of HCV by combined interferon and ribavirin therapy can be achieved in only a minority of cases. On the contrary, severe complications such as glomerulonephritis, sensory-motor neuropathy or diffuse vasculitis can be effectively treated by a combination of corticosteroids, plasma exchange and cyclophosphamide. More recently, a pathogenetic treatment with rituximab, a monoclonal chimeric antibody that binds to the B-cell surface antigen CD20 with selective B-cell blockade, was proposed in patients with HCV-related MC syndrome.
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PMID:[Autoimmune and lymphoproliferative HCV-correlated manifestations: example of mixed cryoglobulinaemia (review)]. 1528 1

The objective of this study was to explore the role of interleukin (IL)-18 in patients with inflammatory myopathies (IM) such as dermatomyositis (DM) and polymyositis (PM) in relation to the possible predominance of a Th1 immune response in their pathogenesis. Serum concentrations of IL-18, interferon (IFN)-gamma, IL-4 and IL-6 were measured in six patients by enzyme-linked immunosorbent assay (ELISA). IL-18 expression was evaluated by in situ hybridization (ISH), whereas CD68, CD8 and CD83 were investigated by immunohistochemistry (IHC) to define the main producers of IL-18. Lastly, the expression of both IL-18 receptor (IL-18R) and monocyte chemoattractant protein (MCP)-1 was also explored by IHC. High serum levels of IL-18 and IFN-gamma, and conversely low titres of IL-4 and IL-6, were demonstrated in both diseases. In addition, IL-18 was overexpressed in muscle biopsy specimens from patients with IM. Both macrophages and dendritic cells (DC) surrounding either perivascular and perimysium areas in DM or endomysium in PM were the main producers of IL-18. Endothelial cells (EC), smooth muscle cells (SMC) and CD8(+) T cells expressed a high content of IL-18R. Vessel cells overexpressed MCP-1 in parallel with IL-18R. High concentrations of serum IL-18 as well as muscular up-regulation of IL-18 and IL-18R suggest that deregulation of the IL-18/IL-18R pathway is a pathogenetic mechanism in IM. Measurement of IL-18 may thus predict the severity of both DM and PM.
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PMID:Interleukin-18 overexpression as a hallmark of the activity of autoimmune inflammatory myopathies. 1696 94


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