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)

The balance between CD4(+) T helper (Th1) lymphocytes producing interferon-gamma or Interleukin-4 (Th2) in the lungs may vary among diseases and during the progression of interstitial pneumonia (IP). Both idiopathic pulmonary fibrosis (IPF) and collagen vascular diseases (CVD) are associated with IP, but the clinical course and the response to treatment are different. Since Th1 or Th2 modulating drugs have been proven to alter the lymphocyte balance in vitro, it is important to elucidate the Th1/Th2 profile in patients with active IP. Bronchoalveolar lavage (BAL) was performed in patients who had IPF (n = 12) or CVD (n = 12) with IP, as well as in patients who had bronchoectasis and bronchopneumonia (n = 12). The CVD patients had rheumatoid arthritis (n = 6), Sjogren's syndrome (n = 2), dermatomyositis (n = 1), progressive systemic sclerosis (n = 2), and CREST syndrome (n = 1) as the underlying diseases. IP activity was evaluated by measuring serum KL-6, which is a clinically useful indicator for IP. The Th1/ Th2 balance and the CD4(+)/CD8(+) ratio were determined for lymphocytes obtained from BAL by flow cytometric analysis. In IPF patients, the CD4(+)/CD8(+) ratio was lower than in CVD patients. IPF patients showed Th2 dominance and CVD patients showed Th1 dominance when IP was active as evaluated by the serum KL-6 level. These data indicated that the Th1/Th2 balance of CD4(+) T cells in the BAL differs between active IPF and CVD, even though KL-6 is elevated in both diseases. Therefore, the Th1/Th2 profile should be investigated to determine the use of Th1/Th2 modulator therapy for active IP with elevation of KL-6.
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PMID:Intracellular Th1/Th2 balance of pulmonary CD4(+) T cells in patients with active interstitial pneumonia evaluated by serum KL-6. 1687 97

Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE), juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDM). Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC) were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal-Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.
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PMID:Increased Fas and Bcl-2 expression on peripheral blood T and B lymphocytes from juvenile-onset systemic lupus erythematosus, but not from juvenile rheumatoid arthritis and juvenile dermatomyositis. 1716 69

The idiopathic inflammatory myopathies, polymyositis (PM) and dermatomyositis (DM), are evaluated as systemic autoimmune diseases without the pathology determined. Past immunohistochemical findings suggested that the effector response is driven predominantly by CD4 T cells and by humoral immunity in DM, and by cytotoxic T cells in PM. However, histological observations of muscle tissue do not necessarily distinguish DM and PM. Thus, the two diseases including amyopathic DM might represent a spectrum of illness in which some patients suffer only from a muscle disease or from a skin disease. In comparison with research studies on other rheumatic diseases, there are much fewer research studies conducted on PM/DM. The relationship between PM and DM is not clear yet. We reviewed past clinical and basic research on the pathology of PM/DM, including research on relevant T cells, B cells and cytokines.
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PMID:[Recent research developments in polymyositis/dermatomyositis]. 1844 10

It is interesting to study an autoimmune condition like dermatomyositis (DM) in the setting of immunosuppression due to human immunodeficiency virus (HIV) infection. An HIV seropositive female aged 30 years, presented with a nonitchy rash over the face, breathlessness, diarrhoea and difficulty in raising her hands above her head. A heliotrope rash around the eyes, Gottron's papules and proximal muscle weakness were found to be present. C reactive protein, erythrocyte sedimentation rate and lactate dehydrogenase levels were raised, but creatinine phosphokinase and anti-nuclear antibody profile were normal. Her HIV serostatus was confirmed by Western blotting, keeping in mind the potential for false positive HIV serology in an autoimmune disorder. Her CD4 count was 379 cells/mm3. An X-ray of the chest showed bilateral pleural effusion with raised pleural fluid adenosine deaminase levels. Clinical findings and laboratory investigations favored the diagnosis of DM and HIV infection with tuberculous effusion in an HIV seropositive patient. She was treated with antibiotics, four-drug anti-tubercular treatment, systemic steroids and later, antiretroviral treatment. Chances of a false positive antibody test for HIV should be considered in a patient having an autoimmune disease such as DM.
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PMID:Dermatomyositis in a human immunodeficiency virus infected person. 1858 92

Infections result in increased mortality rates in patients with polymyositis/dermatomyositis, leading to death in 9 to 30% of cases. The following parameters can be considered of predictive value for infection onset in polymyositis/dermatomyositis: age, lymphopenia, esophageal dysfunction, ventilatory insufficiency, interstitial lung disease, calcinosis cutis, as well as higher mean daily doses of steroids. A great variety of microorganisms may be responsible for pyogenic and opportunistic infections in polymyositis/dermatomyositis. Opportunistic infections are more often due to mycobacteria and fungi (Pneumocystis jiroveci, Candidasp.). Because a great variety of microorganisms may be responsible for opportunistic infections, it seems difficult to initiate primary prophylaxis in patients with polymyositis/dermatomyositis who exhibit risk factors for opportunistic infections. Primary prophylaxis of Pneumocystis jirovecipneumonia should be given in the group of patients exhibiting CD4-cell count lower than 250/mm(3). Vaccination should be performed in patients with polymyositis/dermatomyositis, prior to immunosuppressive therapy institution.
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PMID:[Infections in polymyositis and in dermatomyositis]. 1908 69

Past paradigm that the muscle tissue injury is driven by CD4 T cells and associated humoral immunity in dermatomyositis (DM), and by cytotoxic T cells in polymyositis (PM) is now under challenge. Although pathogenic autoantigens are to be identified, skeletal muscle C-protein was an excellent immunogen to provoke experimental myositis mimicking human PM. Serum antibodies against aminoacyl tRNA synthases appear in PM/DM patients, but more often in interstitial pneumonitis patients. Inflammatory cytokines are obviously involved in the pathogenesis. Animal studies showed that autoimmune myositis occurs without tumor necrosis factor alpha. Indeed, its blockade has yielded inconsistent outcome. Most crucial ones will be therapeutic targets in the future.
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PMID:[Recent advance in polymyositis and dermatomyositis research]. 1928 Sep 27

To clarify the interactions between dendritic cells (DCs) and Th1 and Th17 T cell subsets and the mode of action of IVIG in inflammatory myopathies, Expression of CD4(+) and CD8(+) T cells, immature (CD1a) and mature (DC-LAMP) DCs, interleukin-17 (IL-17) and interferon-gamma (IFN-gamma), was quantified by immunohistochemistry in muscle biopsies from 13 patients (11 with polymyositis (PM) and 2 dermatomyositis (DM)) obtained before treatment with IVIG. The Th1/Th17 cytokine and the immature/mature DC ratio were studied according to the response to IVIG. Immature DCs were rarely detected compared to mature DCs, observed in all samples except one PM. IFN-gamma-producing cell count was higher than IL-17 count. Neither the expression of IFN-gamma nor IL-17 was correlated with that of DC subsets. Seven of the 13 patients (6 PM and 1 DM) responded to IVIG. T cells and DC subsets were not differentially expressed between responders and non-responders. The frequency of IFN-gamma-producing cells was significantly higher in non-responders with an increased IFN-gamma/IL-17-producing-cell ratio. In conclusion, mature rather than immature DC and IFN-gamma-rather than IL-17-producing cells accumulate in inflamed muscle. Increased IFN-gamma-producing cell count and IFN-gamma/IL-17-ratio were found in IVIG non-responders, suggesting a role for the Th17 mediated pathway in the response to IVIG.
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PMID:Th1 and Th17 balance in inflammatory myopathies: interaction with dendritic cells and possible link with response to high-dose immunoglobulins. 1930 20

Patients affected by autoimmune diseases (rheumatoid arthritis (RA), psoriasis, and dermatomyositis) treated with methotrexate (MTX) develop lymphoproliferative disorders (LPDs). These cases have been reported to be diffuse large B-cell lymphoma, Hodgkin lymphoma, or polymorphous post-transplant LPDs. However, angioimmunoblastic T-cell lymphoma (AITL) is extremely rare in the medical literature. In this report, we describe three cases of RA patients who developed MTX-associated LPDs resembling AITL. They developed systemic lymph node swelling after initiation of MTX. The affected lymph nodes showed the histological finding of AITL: polymorphous infiltrates, mainly T-cells and arborizing high endothelial venules. Two cases showed a predominance of CD4-positive cells in proliferative T-cells, whereas the third case showed CD8-positive cells. CD10 was negative in all cases. RNA in situ hybridization of Epstein-Barr virus (EBV) demonstrated EBV-positive B-cells to be scattered in two cases, but not in one case. The lymphoadenopathy spontaneously regressed with cessation of MTX in all three cases, but one case recurred. These are interesting cases of MTX-associated LPDs mimicking AITL, and cessation of MTX is the only cure for patients with MTX-associated LPDs resembling AITL.
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PMID:Methotrexate-associated lymphoproliferative disorders mimicking angioimmunoblastic T-cell lymphoma. 1962 40

Ocular myositis represents a subgroup within the idiopathic orbital inflammatory syndrome, formerly termed orbital pseudotumor. Ocular myositis describes a rare inflammatory disorder of single or multiple extraocular eye muscles. Unilateral or sequential bilateral subacute painful diplopia is the leading symptom of eye muscle myositis. There are at least two major forms, a limited oligosymptomatic ocular myositis (LOOM) with additional conjunctival injections only, and a severe exophthalmic ocular myositis (SEOM) with additional ptosis, chemosis, and proptosis. Eye muscle myositis is an idiopathic inflammation of the extraocular muscles in the absence of thyroid disease, ocular myasthenia gravis, and other systemic, particularly autoimmune mediated diseases, resembling CD4(+) T cell-mediated dermatomyositis. Contrast-enhanced orbital magnetic resonance imaging most sensitively discloses swelling, signal hyperintensity, and enhancement of isolated eye muscles. Typically, corticosteroid treatment results in prompt improvement and remission within days to weeks in most patients. Compiled data of five patients and a review of the clinical pattern, diagnostic procedures, differential diagnoses, and current treatment options are given.
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PMID:Ocular myositis: diagnostic assessment, differential diagnoses, and therapy of a rare muscle disease - five new cases and review. 1966 64

The transcription factor nuclear factor-kappaB (NF-kappaB) is a ubiquitously expressed protein family that is considered crucial in autoimmunity. We describe NF-kappaB p50 and p65, and the inhibitor I-kappaB alpha in the inflammatory exudates characteristic for the different idiopathic inflammatory myopathies (IIM), that is, endomysial CD8(+) cytotoxic T cells invading non-necrotic fibers in polymyositis (PM) and sporadic inclusion body myositis (sIBM), and the perimysial/perivascular CD20(+) B cells and CD4(+) T cells in dermatomyositis (DM). We also analyzed other inflammatory cells in the vicinity of active inflammation sites. Strikingly, actively invading CD4(+) cells in PM and sIBM contained both p65 and p50, whereas I-kappaB alpha was absent. This could point to a high activation state in which these cells are capable of expressing a variety of inflammatory mediators, contributing to the degradation of non-necrotic fibers. Secondly, CD68(+) macrophages in the infiltrates in all three IIM subtypes showed strong nuclear p50 and I-kappaB alpha staining. This may point to a role for p50 in counteracting inflammation, a reaction that could be enhanced by an upregulation of I-kappaB alpha as we observed with immunofluorescence. These results shed further light on the immunopathology of PM, sIBM and DM. CD4(+) and CD68(+) mononuclear cells may play a more prominent role than previously assumed.
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PMID:Distribution of the NF-kappaB complex in the inflammatory exudates characterizing the idiopathic inflammatory myopathies. 1975 75


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