Treatment of systemic sclerosis.
Proper classification of patients into diffuse cutaneous and limited cutaneous subsets and the anticipation of complications are the keys to the management of individuals with systemic sclerosis (scleroderma). Patients with early diffuse disease and rapidly progressive skin thickening are at highest risk to develop serious internal organ involvement (intestine, lung, heart, kidney) and should be considered for disease modifying therapy. The targets of the disease and sites of possible intervention are vascular endothelium (vasoprotection agents), mononuclear cell subsets (immunosuppressive agents), and fibroblasts (colchicine, D-penicillamine). A number of new therapeutic agents with sound scientific rationale are undergoing therapy trials. Much can be done to improve the lifestyle of the scleroderma sufferer. The most dramatic recent development is the ability to reverse kidney involvement with prompt use of angiotensin converting enzyme inhibitors and modern methods of renal dialysis and transplantation. Scleroderma is not a hopeless disease.
Medsger
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TA
Department of Medicine, School of Medicine, University of Pittsburgh, Pennsylvania.
D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010396:Penicillamine / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy*
D007166:Immunosuppressive Agents; D010396:Penicillamine