progressive systemic sclerosis


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progressive systemic sclerosis

progressive systemic sclerosis

Sclerodema An idiopathic connective tissue disorder, characterized by localized or generalized induration of skin due to ↑ collagen deposition in skin, GI tract, lungs, heart, muscle, kidneys; PSS may be confined to skin for long periods of time, but usually encroaches on the viscera, causing malabsorption, respiratory insufficiency before death ensues, usually from heart or renal failure Clinical Raynaud's phenomenon is the initiating event in most Pts; other features include arthralgias, arthritis, early–subcutaneous edema, late–skin induration, skin ulcers, pinched face, dyspnea due to lung fibrosis, cor pulmonale Immunology Autoantibodies–eg, anti-nuclear, anti-centromere, and anti-topoisomerase 1–Scl-70 antibodies Lab Normochromic, normocytic anemia, microangiopathic anemia, polyclonal gammopathy, ↑ ESR Imaging Osteoporosis, interstitial markings in CXR, loss of esophageal and colonic peristalsis, irregular narrowing in renal arteriogram Management CCBs for vasodilation in Pts with Raynaud's phenomenon; penicillamine may slow the disease progression; preventive measures include avoiding tobacco and cold temperature, antibiotics and digitalis as needed Prognosis 5-yr survival, ± 40%. See CREST complex, Raynaud's phenomenon.

progressive systemic sclerosis

See SYSTEMIC SCLEROSIS.
References in periodicals archive ?
The differential diagnosis of progressive systemic sclerosis.
Antinuclear autoantibodies (ANA) are commonly used for screening, diagnosis and monitoring of connective tissue disorders (CTD) such as systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), Sjogren's syndrome (SS), polymyositis (PM) and mixed connective tissue disease (MCTD).

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