Diffuse Systemic Scleroderma

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Abstract
This report concerns 727 patients with systemic scleroderma[long dash]688 (95%) with acrosclerosis (scleroderma with Raynaud''s phenomenon), and 39 (5%) with diffuse scleroderma (without Raynaud''s phenomenon). In diffuse scleroderma, sclerosis usually began centrally and became generalized although acral areas were often spared. Diffuse scleroderma began most often with cutaneous sclerosis of the trunk, but sometimes edema or polyarthritis first indicated disease; acrosclerosis usually began with Raynaud''s phenomenon or peripheral cutaneous sclerosis. In each group, about 65% of patients had esophageal involvement, about 25% had pulmonary sclerosis, and more than 50% had articular involvement. Systemic involvement progressed rapidly in patients with diffuse scleroderma, as illustrated in 2 reported cases. Patients with diffuse scleroderma had a poor prognosis when compared to patients with acrosclerosis Only 4 (17%) of 24 traced patients with diffuse scleroderma, but 271 (74%) of 367 with acrosclerosis, survived more than 5 years. Percentages of patients surviving more than 10 years were similar[long dash]15% of patients with diffuse scleroderma and 62% of patients with acrosclerosis. Diffuse sclerosis almost always progresses rapidly, while acrosclerosis may be acutely progressive or chronic. The only way to predict the course in an individual patient is by observation for 6 months to one year. The demonstration of progressive visceral involvement within this period indicates a poor prognosis.

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