Systemic sclerosis (SSc)The term systemic means that the scleroderma affects the internal organs. The terms limited and diffuse refer to the extent of skin involvement. Systemic sclerosis (SSc) is a chronic multisystem disorder of unknown etiology characterized clinically by thickening of the skin caused by accumulation of connective tissue and by involvement of visceral organs, including the gastrointestinal tract, lungs, heart, and kidneys. The prognosis in limited cutaneous scleroderma is better except for those patients who, after many years, develop pulmonary arterial hypertension or biliary cirrhosis. Involvement of visceral organs may also occur in the absence of any skin involvement, which is referred to as systemic sclerosis sine scleroderma. Preliminary criteria for the classification of systemic sclerosis were developed by the American Rheumatism Association (now called the American College of Rheumatology) for the purpose of uniformity in clinical studies. A major criterion was the presence of sclerodermatous skin changes of the fingers of both hands plus involvement at any location proximal to the metacarpal phalangeal joints, entire extremity, face, neck, chest, and abdomen. Causes of Systemic sclerosis (SSc)Several environmental factors have been associated with the development of SSc and scleroderma-like illnesses. SSc appears to be more common in coal and gold miners, especially in those with more extensive exposure, suggesting that silica dust may be a predisposing factor. Symptoms of Systemic sclerosis (SSc)The outstanding feature of SSc is overproduction and accumulation of collagen and other extracellular matrix proteins, including fibronectin, tenascin, and glycosaminoglycans, in skin and other organs. In the skin, a thin epidermis overlies compact bundles of collagen that lie parallel to the epidermis. In early disease, fingers and hands are swollen. The skin gradually becomes firm, thickened, and eventually tightly bound to underlying subcutaneous tissue (indurative phase). In patients with diffuse cutaneous scleroderma, skin changes will become generalized, involving initially the extremities, followed by the face and trunk over a period of time, varying from months to a few years. In the extremities, the taut skin over fingers gradually limits full extension, and flexion contractures develop. Ulcers may appear on the volar pads of the fingertips and over bony prominences such as elbows, malleoli, and the extensor surface of the proximal interphalangeal joints of the hands. Treatment of Systemic sclerosis (SSc)Many drugs have been used in the treatment of SSc without any consistent or prolonged benefit. In uncontrolled studies, D-penicillamine has been reported to reduce skin thickening and prevent development of significant organ involvement when compared to similar historic controls. Low doses of aspirin block the formation of thromboxane A2, a powerful vasoconstrictor and platelet aggregator. |
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