Progressive Systemic Sclerosis in Auckland: A Ten Year Review with Emphasis on Prognostic Features

Abstract

The clinical records of all patients with scleroderma or Raynaud''s phenomenon who attended hospitals or specialist practice in Auckland during 1970-1979 were examined; 47 patients satisfied the American Rheumatism Association criteria for progressive systemic sclerosis (PSS). Of these, 13 had calcinosis, Raynaud''s phenomenon, sclerodactyly and telangiectasiae (CRST). All but 2 of the 23 surviving patients were interviewed. The incidence of PSS was 6.3/million per yr and no significant difference in incidence was found between Caucasians and Polynesians. Raynaud''s phenomenon was most frequently the presenting symptom. Esophageal involvement was the commonest clinical visceral manifestation. Cumulative survival rates showed that the adverse prognostic features were renal cardiac, and to a lesser extent, pulmonary involvement. Patients with CRST features had a better prognosis and none had renal complications. The natural history of the CRST group was characterized by a long latency between the presenting symptom and the other features of the disease. The recognition of the CRST variant was of retrospective rather than predictive clinical value. In this first clinical study of PSS patients in New Zealand, results of therapeutic intervention were disappointing.