RESUMO
Twenty four patients (20 females, 4 males; aged 10-65 years) with progressive systemic sclerosis have been analysed. Twenty three had proximal scleroderma (14 acrosclerosis, 9 proximal scleroderma). Two patients had CREST syndrome and two had incomplete CREST syndrome. Both patients with CREST syndrome had acrosclerosis; the two with incomplete CREST syndrome had proximal scleroderma. Cutaneous and vasospastic symptoms were the common initial symptoms. Two patients had pulmonary symptoms at the onset. Skin changes (24 cases), Raynaud's phenomenon (20), articular involvement (13) and upper gastrointestinal involvement (12) were the major manifestations during the course of the disease. Three patients had cardiac involvement. Another three had overlap syndrome. Two had clinically manifest renal involvement. Kidney biopsies were abnormal in 5 of 10 clinically normal patients. Three patients died, one each due to involvement of the heart and lung; in the third, the cause of death could not be ascertained.