Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1832588 (PSS)
 2,979 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antibodies to Extractable Nuclear Antigens (ENAs) namely Sm, nRNP, SS-A and SS-B were studied in 397 patients with various connective tissue diseases (CTD), 146 patients with inflammatory polyarthropathies, 16 cases of systemic vasculitides, and 39 normal subjects using counterimmunoelectrophoresis and double immunodiffusion methods. Anti-ENA antibodies were positive in 40.8 percent cases of Systematic lupus erythematosus (SLE) (n = 191), 36.4 percent of overlap CTD (OCTD, n = 44), 27.8 percent of Sjogren's syndrome (n = 18), 10.6 percent of progressive systemic sclerosis (PSS, n = 66) and 2.7 percent of rheumatoid arthritis (n = 111) patients. The correlation of these antibodies with disease features was done. The significant finding was negative association of anti-nRNP antibodies (when present alone) with renal involvement. Anti-Sm antibodies did not correlate with any disease feature. The other associations included correlation of anti-nRNP with pulmonary parenchymal lesions, anti-SS-A with serositis and pulmonary hypertension, and anti-SS-B with myocarditis and recurrent diarrhoea. We conclude that Anti-ENAs may correlate with certain subsets of these diseases but the subject is controversial.
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PMID:Antibodies to extractable nuclear antigens in connective tissue disorders in India: prevalence and clinical correlations. 262 64

This report describes six diffuse scleroderma (PSS) patients with skeletal muscle myositis accompanied by severe myocarditis, diagnosed by CPK-MB elevation in conjunction with severe left ventricular (LV) hypokinesis. Although the myositis improved with steroid therapy in all patients, those treated with steroids alone died due to progressive LV failure. This experience suggests that the LV dysfunction in PSS patients with myositis may have an inflammatory component. Since the myocarditis may not be clinically apparent initially, it is suggested that CPK-MB fractionation and studies of LV function are undertaken in all PSS patients with myositis. The optimal treatment of this disorder, however, has yet to be determined.
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PMID:Myocarditis as a complication in scleroderma patients with myositis. 816 76