Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifteen children with scleroderma have been presented. All had characteristic cutaneous abnormalities at onset and during the course of disease. All were girls. All had visceral involvement, primarily of the gastrointestinal tract, heart, and lungs. The presence of visceral disease might have been missed without specific and extensive diagnostic procedures, including gastrointestinal barium studies, esophageal motility, pulmonary function and carbon monoxide diffusing capacity, and plethysmography. Raynaud's phenomenon was frequent and accompanied by evidence of occlusive vascular disease. Contractures around joints were readily evident and arthralgias were common, but evidence of objective arthritis was absent. Sixty percent of the patients in this series had ANA. Overlap syndromes with myositis and SLE were present in 7 patients. Three of 15 children died 6-10 years after onset of disease.
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PMID:Scleroderma in children. 26 12

We describe ameliorative effects of plasmapheresis in three patients with dermatomyositis-polymyositis resistant to other therapy, particularly prednisolone. Serologically, the cases were marked, respectively, by a weakly-reactive speckled-type ANA; a strongly reactive speckled-type ANA and an unidentifiable precipitin line by CIEP; and an antibody to cytoplasm and to Jo1. The remission induced by plasmapheresis was temporary in one case and lasting, with low doses of prednisolone, in two. The nuclear autoantibody in case two became undemonstrable after plasmapheresis but recurred during a subsequent relapse. This experience with plasmapheresis in myositis supplements a substantial reported experience of benefit from this therapy.
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PMID:Plasma exchange in dermatomyositis/polymyositis: beneficial effects in three cases. 324 87

Myositis in childhood is characterized by elevated serum levels of muscle-derived enzymes, proximal symmetrical muscle weakness, abnormal EMG findings, and a muscle biopsy, which frequently documents an inflammatory process. In the pediatric age group, JDMS, which has characteristic cutaneous involvement in addition to myositis, is much more common than PM and is more common among female patients. With the use of steroids, mortality has been reduced from 33 per cent to 7 per cent. The development of calcifications can be the most debilitating consequence of JDMS. It is our premise that JDMS is a distinct disease entity and that the increase in HLA-B8 and DR3 in JDMS suggests that genetic background may predispose to disease development. There are conflicting data concerning immunologic abnormalities in JDMS, but there appears to be impairment of natural killing and evidence of complement activation. Results of tests for ANA frequently are positive in JDMS, but Jo-1 antibody, found in some adults with PM, has not been found in JDMS. Most newly diagnosed JDMS patients have antibodies to coxsackie B that may be related to the pathogenesis of this disease. Specific pathologic findings of endothelial cells containing reticulotubular inclusions are associated with small vessel occlusion, subsequent obliteration, and increased factor VIII levels in clinically active disease. In addition to physical therapy, steroids are used most frequently, but other immunosuppressive agents and plasmapheresis have been tried in severely ill children. Rigorous evaluation of the efficacy of these modalities is needed.
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PMID:Juvenile dermatomyositis. 353 4

Myositis in childhood is characterized by elevated serum levels of muscle-derived enzymes, proximal symmetrical muscle weakness, abnormal EMG and a muscle biopsy which frequently documents an inflammatory process. In the paediatric age group, JDMS is much more common than PM and occurs more frequently among females. Mortality has been reduced from 33 per cent to 7 per cent following the use of steroids. The development of calcifications (33 per cent) can be the most debilitating consequence of JDMS . It is our premise that JDMS is a distinct disease entity and that the increase in HLA-B8 and -DR3 in JDMS places this disease in the company of other immunopathic disorders. There are conflicting data concerning immunological abnormalities in JDMS , but there appears to be impairment of natural killing and evidence of complement activation. The frequent positive ANA in JDMS raises the speculation of its relationship to the antinuclear antibody, Jo-1, found in some adults with PM, which has specificity for tRNAHis. Most newly diagnosed JDMS patients have antibodies to Coxsackie B which may be related to the pathogenesis of this disease. Specific pathological findings of endothelial cells containing reticulotubular inclusions are associated with vessel occlusion, subsequent obliteration and increased Factor VIII levels in clinically active disease. In addition to physical therapy, prednisone is the drug most used, but immunosuppressive agents and plasmapheresis have been tried in severely ill children. Rigorous evaluation of the efficacy of these modalities is needed.
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PMID:Juvenile dermatomyositis and polymyositis. 673 23

Thirty-two patients with polymyositis were categorised into 4 groups: (1) 'pure' polymyositis, (2) dermatomyositis, (3) myositis associated with autoimmune 'overlap syndrome', and (4) those with associated malignancy. Serum from each patient was examined for a range of antinuclear antibodies. Seventeen patients had ANA detected by immunofluorescence, 18 patients had raised DNA binding (greater than 25 U/ml), of whom eight had levels greater than 50 U/ml (SI conversion: U/l = U/ml x 10(3)). Antibodies to soluble nuclear antigens were detected in 23 (72%) by 1 or more of 3 methods, and in all of these anti-RNP was the main antibody detected. Antibodies to other soluble antigens were also present in 6 sera. In 2 cases, both patients with SLE/myositis overlap, these were shown to be anti-Sm. The remaining 4 had antibodies to various protein components of the extracts, but it was not possible to demonstrate an antibody of diagnostic specificity for polymyositis. Furthermore, quantitation of anti-RNP and anti-DNA antibodies failed to define a distinct clinical entity or exclude malignant disease. High levels of anti-RNP antibodies showed an association with Raynaud's phenomenon, sclerodactyly, and pulmonary fibrosis and an inverse correlation with the rash of dermatomyositis, suggesting that this antibody may be of pathogenetic rather than diagnostic significance.
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PMID:Antibodies to nuclear antigens in polymyositis: relationship to autoimmune 'overlap syndromes' and carcinoma. 678 93

A 57-yr-old lady developed polymyositis whilst taking D-penicillamine for RA. D-Penicillamine-induced polymyositis occurs in RA with a greater frequency than idiopathic polymyositis. Anti-acetyl choline receptor antibodies and ANA were positive, consistent with drug-induced disease. Anti-Jo-1 antibodies are considered specific for idiopathic myositis, and their presence was unexpected. Following withdrawal of the drug, the disappearance of the anti-Jo-1 and other antibodies coincident with clinical improvement, suggested that D-penicillamine was responsible for inducing antibody production.
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PMID:D-penicillamine and polymyositis: the significance of the anti-Jo-1 antibody. 825 25

Clinical characteristics and immunoserological features of patients with overlap myositis treated in Clinic for Rheumatology of Military Medical Academy in the period 1978-1996 are presented in the study. Retrospective and partially prospective study included 16 patients with the diagnosis of dermatopolymyositis associated with some other systemic disease of connective tissue (SDCT) in overlap syndrome. There were 12-females of average age 37.33 years and 4 males of average age 52.25 years. Complete laboratory investigation included the determination of muscular enzymes values. Immunoserological analyses were also performed (RF, Le cells, ANA, ICs, immunoglobulins, cryoglobulins and complement), EMNG examination of pelvis and shoulder and pH examination of skin, subcutaneous tissue and muscles. The combination of the diseases was found in 11 patients, 7 were with PSS, 2 with SLE, 1 with RA and one with PAN. Three diseases were associated in 4 patients, SLE and RA in 2, PSS and RA in 1, thyroiditis and sicca complex in 1. Five-fold combination of disease was found in 1 patient. More frequent occurrence of fever, polyarthritis, skin manifestations, Raynoud's phenomenon, the involvement of serosas, lungs and heart, histological finding of skin and/or muscle vasculitis and larger presence of ANA, ICs, hypergammaglobulinemia and hypocomplementemia pointed out the dominant disorder of humoral immunity which was most probably connected with associated SDCT.
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PMID:[Dermatopolymyositis and overlap syndromes]. 961 23

Patient complaints arising as manifestations of medication side effects are commonly encountered in clinical practice. A rheumatologist must routinely consider side effects of drugs in the differential diagnosis of many symptoms. This review will remind the reader of certain well-described and some newly reported side effects commonly encountered in an internal medicine practice. Focal points incude arthralgias/arthritis, myopathy/myositis, ANA/drug-induced lupus, bone loss/osteoporosis, and tendon rupture.
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PMID:Rheumatic manifestations of current pharmacopeia. 1112 52

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

The strongest predictive factor for the development of interstitial lung disease (ILD) in myositis (IIM) patients is the presence of different antisynthetase antibodies. The aim of this study was to compare the clinical characteristics, radiological findings and therapeutic response between the anti-SS-A positive and negative antisynthetase syndrome (ASS) patients. A prospective study of 315 IIM patients was conducted including 27 anti-Jo-1 positive ASS patients. Mean disease duration was 46.6 (range 4-198) months. All patients fulfilled the classification criteria for IIM. All patients underwent chest radiography, pulmonary function tests and HRCT at he time of diagnosis and 6 months after the immunosuppressive therapy. Routine laboratory tests, RF, ANA, anti-ENA, anti-SS-A, anti-histidyl-transfer RNA antibody (Jo-1) measurements were performed in all patients. ILD was found to be present in 70.4% of ASS patients. The anti-SS-A negative ASS group had a more frequent association with alveolitis and responded well to immunosuppressive therapy (p < 0.05). HRCT scan showed more fibrosis in the SS-A positive group. 15.8% of patients died due to pulmonary or cardiac complications. In conclusion, coexistence of anti-SS-A and anti-Jo-1 antibody may be a good predictor for a more coarse and severe ILD in IIM patients who require a more aggressive approach in therapy.
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PMID:Characteristics of interstitial lung disease in SS-A positive/Jo-1 positive inflammatory myopathy patients. 1926 2


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