progressive systemic sclerosis


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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 ?
Pulmonary disease in progressive systemic sclerosis: a complication of gastroesophageal reflux and occult aspiration?
From the result shown in fig.1, it is quite apparent that patients with SLE and other rheumatic diseases such as rheumatoid arthritis (RA), mixed connective tissue disease (MCTD), progressive systemic sclerosis (PSS) exhibited a significant decrease in the haemoglobin content compared to normal control (P< 0.001 for each, except RA P < 0.05), while in the other rheumatic diseases [Polymyositis (PM), ankylosing spondylitis (AS)], its level did not show any significant changes.
Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma.
Manometric assessment of esophageal involvement in progressive systemic sclerosis, morphoea and Raynaud's disease.
Kawakami, "Association of progressive systemic sclerosis with pulmonary sarcoidosis.
The differential diagnosis of progressive systemic sclerosis. Bull Rheum Dis 1981; 31: 7-10.

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