progressive systemic sclerosis


Also found in: Dictionary, Thesaurus, Acronyms, Encyclopedia.

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.

progressive systemic sclerosis

; PSS systemic disease of autoimmune origin, characterized by hyalinization and fibrosis of dermal and mesodermal collagen; skin becomes indurated and tied down to underlying structures, digital pulps atrophy and ulcerate; strong association with Raynaud's disease or other autoimmunopathies, e.g. renal dysfunction (see scleroderma; sign, Lhermitte's)
References in periodicals archive ?
Connective tissue disorders such as progressive systemic sclerosis, systemic lupus erythematosus, and dermatopolymyositis are secondary causes of intestinal pseudo-obstruction.
ABSTRACT: A female patient with progressive systemic sclerosis and pulmonary fibrosis had a dermatofibrosarcoma protuberans on the right thigh.
A 63-year-old woman had had progressive systemic sclerosis with pulmonary fibrosis since 1986 and had been treated over the years with cyclosporine and prednisolone.

Full browser ?