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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A syndrome of acquired immunodeficiency has been identified in a group of rhesus monkeys (Macaca mulatta) which died at the California Primate Research Center. Clinical evaluation of these animals revealed that 50% or more had lymphadenopathy, weight loss, and diarrhea. At least 30% had splenomegaly, fever, cutaneous abscesses and/or arthritis/myositis. Two animals had fibrosarcomas. Anemia was seen in 19 animals, lymphopenia in 14, granulocytopenia in four and thrombocytopenia in three. Hepatitis was diagnosed histopathologically in 13. Electrophoresis revealed hypoproteinemia, hypoalbuminemia and hypogammaglobulinemia. Numerous bacterial, protozoal, and viral agents were identified including cytomegalovirus and leukocyte-associated herpesvirus. Pathologic lesions included severe post-reactive depletion of lymphocytes in germinal centers and paracortical regions of lymph nodes. Clinical and pathologic changes indicate an acquired immunodeficiency syndrome which has some similarities to AIDS in humans. This disease in monkeys may provide a model for studying that disease.
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PMID:Clinical features of simian acquired immunodeficiency syndrome (SAIDS) in rhesus monkeys. 632 13

Juvenile dermatomyositis (JDMS) is a systemic vasculopathy characterized primarily by inflammation of skin and muscle. JDMS is identified in more than three per million persons per year, using established diagnostic criteria. Although originally thought to be a relatively homogeneous disease, new data confirm that heterogeneity in JDMS may be found at several levels and that each variant may be associated with a different disease course. Unlike adults with dermatomyositis, of whom more than 50% have a specific myositis-associated antibody (MSA), a much smaller number of children appear to test positive for a known MSA (about 10%), despite the evidence that more than 60% of children with JDMS test positive for antinuclear antibodies. In children, the most common MSA is directed against Mi-2, not toward one of the tRNA synthetases, such as tRNA histidine, as is found in 20% to 30% of adults with myositis. About 50% of children with JDMS have circulating evidence of endothelial cell damage (increased vWF:Ag), whereas others have different indicators of disease activity, such as elevated neopterin (> 60%) or increased circulating B cells with peripheral lymphopenia (> 80%). Newer modes of assessment of functional ability may help evaluate response to therapy. Finally, physicians with newly diagnosed (< 6 months) JDMS patients are urged to call the new National Institutes of Health Rare Disease Registry for New Onset Dermatomyositis (312-880-3333) to enroll their patients and for more information on the onset of this disease.
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PMID:An update on juvenile dermatomyositis. 851 18

A Danish multicentre study was undertaken of the manifestations, infections, thrombotic events, survival and predictive factors of survival in 513 Danish patients with systemic lupus erythematosus (SLE) according to the 1982 classification criteria of the American College of Rheumatology. The mean duration of follow-up was 8.2 years from diagnosis and 12.8 years from first symptom. This paper describes the most common clinical and laboratory manifestations and their relationship to sex and age at the time of onset and diagnosis. Cluster analysis revealed three clinically defined clusters at the time of disease onset. Cluster 1 (57% of patients) consisted of relatively elderly patients without nephropathy or malar rash, but with a high prevalence of discoid lesions. Cluster 2 (18%) consisted of patients with nephropathy, a third of whom also developed serositis and lymphopenia. The patients of the third cluster (25%) all had malar rash and half were photosensitive. Follow-up showed that the patients of cluster 2 developed azotaemia, large proteinuria, arterial hypertension and myositis significantly more often than did the rest of the patients, but the mortality was not increased. The risk of developing renal end-stage disease was highest in men with early-onset disease.
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PMID:A multicentre study of 513 Danish patients with systemic lupus erythematosus. I. Disease manifestations and analyses of clinical subsets. 989 Jun 74

Several extrahepatic manifestations have been associated with infection with Hepatitis C virus (HCV) infection. It has been associated with Sjogren's syndrome (SS) and inflammatory myositis (IM). The objective was to look at the prevalence of anti-HCV antibodies in the serum of SS and IM patients of Indian origin. Individuals satisfying the European Economic Community criteria for the diagnosis of SS and those satisfying the criteria of Bohan and Peter for the diagnosis of IM were recruited in the study. Routine evaluation for liver functions was made. Anti-HCV antibodies were tested by a third generation ELISA, using microplate HCV3.0 ELISA. Of the 23 patients with SS studied, 14 had extraglandular features. The commonest were anaemia and arthritis in six each, followed by in lymphopenia in two. One patient each had interstitial lung disease, hypothyroidism and chronic active hepatitis. Twenty-two patients with IM were studied alongside. None of the patients had abnormal liver functions. One patient with primary SS tested positive for anti-HCV antibodies. None of the patients with inflammatory myositis tested positive for anti-HCV antibodies. The presence of anti-HCV antibodies in our cohort of patients with SS and IM is low and more in keeping with the generally low prevalence of the infection in the Indian population.
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PMID:Paucity of anti-hepatitis C virus antibodies in the serum of Indian patients with Sjogren's syndrome and inflammatory myositis. 1502 6

The association of systemic lupus erythematosus (SLE) with idiopathic polymyositis or dermatomyositis is reported to occur in the range of 4-16%. Myositis can occur before or after SLE, or sporadically both diseases can be present simultaneously. This case report concerns a 36-year-old female patient suffering from Raynaud's phenomenon, polyarthralgia in the small joints of the hands, and skin changes compatible with Gotron's indications. Symmetric proximal muscle weakness of the extremities, fever of up to 40 degrees C, heliotrope rashes with erythematous changes in the face, upper arms, and posterior shoulders occurred subsequently. Laboratory analyses revealed increased acute phase reactants, hypochromic anaemia, lymphopenia, and increased levels of all muscle enzymes. Immunoserology demonstrated positive ANA, anti-Sm, and anticardiolipin antibodies (aCL), while anti dsDNA, anti Ro, anti La, and anti Jo-1 antibodies proved negative. Hypocomplementaemia and elevated levels of immune complexes were also detected. Pathologic sediment and proteinuria were revealed via urine analyses, while a kidney biopsy confirmed lupus nephritis (type IVa according to the World Health Organisation classification). Biopsy of erythematous changes of the posterior shoulder demonstrated leukocytoclastic vasculitis. Electromyography of the lower extremities established myopathic changes. Inflammation of the muscles was confirmed via magnetic resonance imaging. The patient was categorised as having two separate coexistent diseases--SLE and dermatomyositis. Both the classification criteria of the American College of Rheumatology for SLE and the diagnostic criteria for dermatomyositis, proposed by Bohon and Peter, were fulfilled simultaneously. Treatment commenced with pulses of methylprednisolone and continued with oral therapy, including Resochin. Pulses of intravenous cyclophosphamide were also administered. After six weeks of therapy, biohumoral remission of both diseases was achieved, while complete recovery from muscle weakness was accomplished after four months.
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PMID:[Systemic lupus erythematosus and dermatomyositis--case report]. 1653 99

STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disorder characterized by blood vessel occlusions, acral necrosis, myositis, rashes, and pulmonary inflammation that are the result of activating mutations in the STimulator of Interferon Genes (STING). We generated a transgenic line that recapitulates many of the phenotypic aspects of SAVI by targeting the expression of the human STING-N154S-mutant protein to the murine hematopoietic compartment. hSTING-N154S mice demonstrated failure to gain weight, lymphopenia, progressive paw swelling accompanied by inflammatory infiltrates, severe myositis, and ear and tail necrosis. However, no significant lung inflammation was observed. X-ray microscopy imaging revealed vasculopathy characterized by arteriole occlusions and venous thromboses. Type I interferons and proinflammatory mediators were elevated in hSTING-N154S sera. Importantly, the phenotype was prevented in hSTING-N154S mice lacking the type I interferon receptor gene (Ifnar1). This model, based on a mutant human STING protein, may shed light on the pathophysiological mechanisms operative in SAVI.
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PMID:Expression of a constitutively active human STING mutant in hematopoietic cells produces an Ifnar1-dependent vasculopathy in mice. 3122 25

Polymyositis is a diagnosis of exclusion. In patients with odd features, it can be of infective etiology. A high index of suspicion is required for diagnosis. A 55-year-old gentleman presented with gradual-onset proximal muscle weakness. Examination revealed mild distal weakness but no rash. Muscle enzymes were raised and tests for autoantibodies were negative. Biopsy revealed microsporidiosis. In view of this unusual infection, immunodeficiency was considered and he was found to have lymphopenia which antedated his illness. Later, he developed cranial nerve palsies due to multiple lesions in the pons. In addition, he had Cytomegalovirus viremia. Literature was reviewed to identify 20 cases of microsporidial myositis, its presentation, underlying immunodeficient state, and clinical course. Infective polymyositis should be considered in a patient with paucity of clinical and serological autoimmune features. Lymphopenia can point to underlying immunodeficiency. CMV infection could be the contributor to or bystander-effect of idiopathic lymphopenia.
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PMID:Microsporidial myositis in adult-onset immunodeficiency: case-based review. 3150 96

Idiopathic inflammatory myopathies (IIM) are a group of rare connective tissue diseases (CTDs) deeply affecting patients' prognosis. Extra-muscular involvement is not rare and skin, joints and lung are the most common targets. However, also dyserythropoiesis has been described, carrying relevant issues on patients' management and follow-up, as for example, lymphopenia has been associated with an increased risk of rapid progressive interstitial lung disease in anti-MDA5 positive dermatomyositis. Conversely to systemic lupus erythematosus, thrombocytopenia has only been rarely described in IIM and very few authors focused on its potential prognostic implications. We describe five cases of thrombocytopenia in IIM patients positive for myositis specific (MSA) or associated (MAA) autoantibodies. These reports extend the spectrum of haematological features associated to IIM, focusing also on potential risk factors for thrombocytopenia occurrence.
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PMID:Thrombocytopenia in idiopathic inflammatory myopathies: a case series analysis. 3182 Jul 22