Progressive systemic sclerosis in Auckland: a ten year review with emphasis on prognostic features

The clinical records of all patients with scleroderma or Raynaud's phenomenon who attended hospitals or specialist practice in Auckland during the ten year period of 1970--79 were examined. Forty-seven patients were found to satisfy the American Rheumatism Association criteria for progressive systemic sclerosis (PSS). Thirteen of these patients had CRST features which comprise calcinosis, Raynaud's phenomenon, sclerodactyly and telangiectasiae. All but two of the 23 surviving patients were interviewed. The incidence of PSS was 6.3/million/year and no significant difference in incidence was found between Caucasians and Polynesians. Raynaud's phenomenon was most frequently the presenting symptom. Oesophageal 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 the CRST features had a better prognosis and none had renal complications. The natural history of the CRST group was characterised by a long latency between the presenting symptom and the other features of the disease. Therefore, 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.