Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary Sjogren's syndrome (pSS) is a chronic autoimmune disorder of the exocrine glands. The diagnosis is largely based on keratoconjunctivitis sicca and xerostomia in the presence of anti-SS-A and/or SS-B antibodies. Anticentromere antibodies (ACA) have occasionally been reported in patients with pSS. We describe two patients with pSS associated with ACA, initially diagnosed as limited systemic sclerosis. Symptoms at the time of initial presentation were dry eyes and mouth, arthralgias, and Raynaud's phenomenon. Both patients developed small vessel cutaneous vasculitis, parotid enlargement, low C4 complement levels, positive rheumatoid factor, and lymphoma. These findings suggest that patients with pSS who have ACA may be a subgroup of patients at increased risk of extraglandular systemic manifestations and lymphoma.
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PMID:Primary Sjogren's syndrome with anticentromere antibodies--a clinically distinct subset. 2008 42

Pulmonary artery hypertension is an infrequent accompaniment of rheumatologic diseases. It could result as a complication of diseases like systemic sclerosis (SSc), systemic lupus erythomatosus (SLE), and mixed connective tissues diseases (MCTD). Very rarely, rheumatoid arthritis, dermatomyositis and Primary Sjogren's syndrome can also be complicated by a rise in pulmonary artery pressure. Pulmonary artery hypertension (PAH) can also be found in many other non rheumatologic diseases. Right heart catheterization (RHC) is the gold standard investigation in diagnosing pulmonary hypertension. This article aims to discuss the role of RHC in diagnosing pulmonary hypertension, its comparison with other modalities, the procedure, and its role in treatment of connective tissue disorders, especially with a special reference to the Indian context.
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PMID:Role of Right Heart Catheterization in Rheumatologic Diseases - The Indian Perspective. 3133 Nov 41