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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the course of a systematic study of T cell lines derived from muscle of patients with various inflammatory myopathies, we identified a new form of polymyositis that is mediated by gamma-delta T cells. In the affected patient's muscle CD3+CD4-CD8- gamma-delta T cells surrounded and invaded nonnecrotic muscle fibers in the same way as CD3+CD8+ alpha-beta T cells surround and invade nonnecrotic muscle fibers in inclusion body myositis and other forms of polymyositis. Gamma-delta T cells were extremely rare or absent in muscles and muscle-derived T cell lines in other patients with polymyositis, inclusion-body myositis, dermatomyositis or granulomatous myopathy. This new form of polymyositis has provided us with a unique opportunity to study cytotoxic gamma-delta T cells and their muscle-fiber targets in situ. All muscle fibers expressed HLA-class I antigen and the 65-kD heat-shock protein. The autoaggressive behavior of the gamma-delta T cells is consistent with the hypothesis that in some inflammatory myopathies autoinvasive T cells recognize muscle fiber associated antigen(s). Further studies are needed to define the type of gamma-delta T cell receptor used and the antigen(s) recognized by gamma-delta T cells in this rare type of autoimmune muscle disease.
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PMID:The role of gamma-delta T lymphocytes in inflammatory muscle disease. 153 37

Twenty five patients with idiopathic myositis attended this department for long term follow up from 1980 to 1989. Twelve patients had primary polymyositis (four men, eight women) and six had primary dermatomyositis (three men, three women); five women had an overlap syndrome. Two patients had a malignant condition associated with the myositis. The mean age at diagnosis was 40 years. All of the patients had proximal muscle weakness, 18/25 had a raised creatine kinase value (mean 2325 IU/l), 19/20 had an abnormal electromyogram, and 19/24 had positive muscle biopsy samples. Of the disease specific antibodies, anti-Jo-1 was detected in only 1/21 patients tested (three patients with fibrosing alveolitis were negative for this antibody), but the 56 kDa antibody was detected in 12/17 patients. The HLA data analysed in the white patients (17/25) showed that 6/8 of those tested were HLA-DR3 positive. All patients were treated with prednisolone and azathioprine was used for 14/25 patients. Only three deaths occurred during the eight year follow up, but there was a substantial morbidity, which may reflect the referral pattern. Muscle strength tests and creatine kinase levels were useful in recording the response to treatment in some patients. These data emphasise that careful long term follow up of patients with myositis is mandatory and that although the present treatment strategy has substantially reduced the death rate, morbidity associated with the disease remains a major problem.
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PMID:Idiopathic myositis: a rheumatological view. 154 36

The IIM are a heterogeneous group of systemic rheumatic diseases which share the common features of chronic muscle weakness and mononuclear cell infiltrates in muscle. A number of classification schemes have been proposed for them, but none takes into consideration the marked immunologic, clinical, and genetic heterogeneity of the various clinical groups. We compared the usefulness of myositis-specific autoantibodies (anti-aminoacyl-tRNA synthetases, anti-SRP, anti-Mi-2 and anti-MAS) to the standard clinical categories (polymyositis, dermatomyositis, overlap myositis, cancer-associated myositis, and inclusion body myositis) in predicting clinical signs and symptoms, HLA types, and prognosis in 212 adult IIM patients. Although patients with inclusion body myositis (n = 26) differed in having significantly more asymmetric and distal weakness, falling, and atrophy than other patients, there were few other significant differences among the other clinical groups. In contrast, autoantibody status defined distinct sets of patients and each patient had only 1 myositis-specific autoantibody. Patients with anti-amino-acyl-tRNA synthetase autoantibodies (n = 47), compared to those without these antibodies, had significantly more frequent arthritis, fever, interstitial lung disease, and "mechanic's hands"; HLA-DRw52; higher mean prednisone dose at survey, higher proportion of patients receiving cytotoxic drugs, and higher death rates. Those with anti-signal recognition particle antibodies (n = 7) had increased palpitations; myalgias; DR5, DRw52; severe, refractory disease; and higher death rates. Patients with anti-Mi-2 antibodies (n = 10) had increased "V-sign" and "shawl-sign" rashes, and cuticular overgrowth; DR7 and DRw53; and a good response to therapy. The 2 patients with anti-MAS antibodies were the only ones with alcoholic rhabdomyolysis preceding myositis; both had insulin-dependent diabetes mellitus, and both had HLA-B60, -C3, -DR4, and -DRw53. These findings suggest that myositis-specific autoantibody status is a more useful guide than clinical group in assessing patients with myositis, and that specific associations of immunogenetics, immune responses, and clinical manifestations occur in IIM. Thus the myositis-specific autoantibodies aid in interpreting the diverse symptoms and signs of myositis patients and in predicting their clinical course and prognosis. We propose, therefore, that an adjunct classification of the IIM, based on the myositis-specific autoantibody status, be incorporated into future studies of their epidemiology, etiology, and therapy.
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PMID:A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogeneous patient groups. 165 47

Dermatomyositis is a disease of unknown etiology characterized by progressive, symmetrical, proximal muscle weakness with accompanying compatible cutaneous findings. Thirty-nine patients with dermatomyositis from the Louisville, Kentucky area were enrolled in this study. Patients were grouped into those with or without a malignancy. Ten patients (26%) either had or have had a malignancy. Twenty-five Caucasian patients were HLA typed for the A, B, DR and DQ locus antigens, of whom 5 had an associated malignancy and 20 did not have a malignancy. We found that no single antigen had a significantly increased or decreased frequency as compared with our control population for the entire group, or for any clinical subset we examined. Serologic testing revealed 4 patients with anti-Mi-2 antibodies and 1 patient with anti-PM-SCL antibodies. No patient had a positive anti-Jo-1 antibody in this group. The results of serologic tests in this group did not correlate with any clinical subset or HLA antigen. Our findings were in agreement with the previous reports in which approximately 25% of patients with DM have an associated malignancy. Our findings also support the notion that untargeted malignancy searches are not warranted. Contrary to previous reports we did not observe an inverse relationship between cancer and pulmonary disease in the dermatomyositis patient. This study does not indicate that there are any HLA associations or clinical associations, other than age, that distinguish patients with dermatomyositis as running a greater risk of developing malignancy.
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PMID:Clinical, serologic, and immunogenetic studies in patients with dermatomyositis. 168 47

Antinuclear antibodies (ANA), as detected by indirect immunofluorescence on HEp-2 cells, have been investigated in five spouses and 41 first-degree relatives of nine probands with polymyositis-dermatomyositis (PM-DM) and in 41 sex- and age-matched controls. ANA were detected in 12 out of the 41 first-degree relatives and in two controls (chi 2 = 6.97; P less than 0.01). HLA typing was done in four out of the nine families; in two of them only, ANA segregated with a haplotype. ANA positivity was not correlated either to sex or to age or to household contact. Our results show that ANA occur in a significant percentage of first-degree relatives of patients with PM-DM. The finding seems to be genetically conditioned.
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PMID:Antinuclear antibodies in first-degree relatives of patients with polymyositis-dermatomyositis: analysis of the relationship with HLA haplotypes. 174 97

A case of dermatomyositis-like syndrome is described in a 19-year-old man with a history of Bruton's hypogammaglobulinemia. Although the patient had central-nervous-system manifestations (seizures), no echovirus was isolated in the cerebrospinal fluid, in contrast to previously reported cases. Data for our case and the 15 cases previously reported in the literature are reviewed. HLA typing of our patient revealed the presence of HLA B8 and DR3, which seems to play a major role in juvenile dermatomyositis.
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PMID:Dermatomyositis-like syndrome in X-linked hypogammaglobulinemia. Case-report and review of the literature. 197 55

A review of patients presenting at the rheumatology clinic of the Parirenyatwa Hospital, University of Zimbabwe School of Medicine, revealed 14 with HIV infections. Over a 6-month period, 141 patients had been diagnosed with rheumatic diseases, including 49 with rheumatoid arthritis, 18 with systemic lupus erythematosus (SLE), 5 with dermatomyositis and 3 with scleroderma. Rheumatic diseases were thought to be rare in this population, of whom only 0.2% carry the HLA B27 antigen. Recently a marked increase in patients with reactive or Reiter-like illness, the most common arthropathy in HIV+ patients, were referred. These 14 patients, mostly males, all had acute onset arthropathy, 5 with polyarthritis and 9 with oligoarticular diseases, usually of the knees and ankles, usually symmetrical, or asymmetrical in the small peripheral joints. Synovial fluid was negative except for leukocytosis. The duration of the illness was usually 3-6 months. In addition there were 3 HIV+ patients with complete Reiter's and 7 HIV+ with incomplete Reiter's syndrome, out of a total of 16 Reiter's patients. Among the associated symptoms were urethritis, cervicitis, conjunctivitis, balanitis and oral ulceration, but not psoriasis. These patients had elevated sedimentation rates, but otherwise negative blood findings, other than anemia. In contrast 36 patients with rheumatoid arthritis and 12 with SLE were HIV-. 2 HIV patients also had septic arthritis, a common condition in Zimbabwe.
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PMID:Human immunodeficiency virus-related connective tissue diseases: a Zimbabwean perspective. 204 91

We describe 2 patients with dermatitis herpetiformis who developed polymyositis/dermatomyositis. On HLA typing, both patients were found to be HLA-B8, DR3 positive. The concurrence of these two relatively rare diseases, both associated with immunologic abnormalities, further supports the role of autoimmunity in their pathogenesis and indicates a possible common genetic basis. It also suggests that myositis may be more common in patients with dermatitis herpetiformis than in the general population.
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PMID:Polymyositis/dermatomyositis associated with dermatitis herpetiformis. 278 48

D-penicillamine (DPA) leads to side effects in different ways: collagen and elastin crosslinking are inhibited, which results in thin and vulnerable skin, cutis laxa, elastosis perforans serpiginosa, wound healing defects and embryopathy. Toxic influences effect thrombo- and leukocytopenia (incidence 5-15%), gastrointestinal disturbances (10-30%), changes or loss of taste (5-30%), loss of hair (1-2%), and partly proteinuria (5-20%). Acute hypersensitive reactions include DPA-allergy (2-10%). Severe adverse effects are autoimmune phenomena such as pemphigus, DPA-induced lupus erythematosus, polymyositis/dermatomyositis, membranous glomerulopathy and hypersensitivity pneumonitis (like Good-pasture's syndrome) and myasthenia (all less than 1%). In addition there are a number of rare side effects, often single observations. Risk factors include a genetic disposition (especially HLA-B8 and -DR3), poor sulphoxidizers and, to a certain degree, higher age. During pregnancy and in clinically relevant disturbances of bone marrow, liver and renal function DPA is contraindicated. The total incidence of side effects amounts to 30-60%, the withdrawal rate is 20-30%; therefore clear indications and a regular survey of DPA therapy are necessary.
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PMID:[D-penicillamine--side effects, pathogenesis and decreasing the risks]. 306 3

Twenty probands with juvenile dermatomyositis and their relatives were studied to determine the inherited segregation patterns of class I, II, and III HLA region markers including C4A, C4B, Bf, and C2 complement polymorphisms. The extended haplotype B8, DR3, C4A*Q0, C4B*1, C2*C, and Bf*S was present in 13 of the 20 probands. Three other probands also carried a haplotype with a null allele for C4A and two further probands carried a null allele for C4B; only two probands had no detectable C4 null allele. These data confirm previous studies showing high frequencies of B8 and DR3 in patients with juvenile dermatomyositis, but show that there is a higher association with null alleles of C4. This suggests that the C4 genes are either themselves the disease-susceptibility genes or are in very strong linkage disequilibrium with such genes.
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PMID:C4 complement allotypes in juvenile dermatomyositis. 326 May 84


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