SOUTH AUSTRALIAN SCLERODERMA REGISTER - ANALYSIS OF DECEASED PATIENTS

The demographic, clinical, pathological and serological features of 12 3 deceased patients with systemic sclerosis have been analysed. These patients consisted of all identified patients dying with this disease in South Australia between 1983 and 1996 inclusive. There were 85 fema les and 38 males, with the ratio of limited:diffuse:overlap disease su bset being 9:5:1. Disease characteristics revealed that patients with the limited disease tended to be female with high frequencies of the c entromere autoantibody, while patients with the diffuse disease had eq ual gender representation with the frequent presence of nucleolar, spe ckled or homogeneous antinuclear antibody. Mean duration of disease an d mean age of death for the limited:diffuse:overlap subsets differed s ignificantly between groups (p<0.05) and were 16.5, 9.3 and 10.9 years and 71.9, 57.8 and 52.8 years respectively. Cumulative survival curve s for the subsets differed highly significantly, with patients with th e limited diseases dying more commonly from right heart failure (docum ented terminally in 25% of the centromere positive limited subset), ca rdiovascular disease or cancer, while patients with the diffuse subset died from respiratory failure, renal failure or cardiovascular diseas e. In conclusion, this retrospective analysis has revealed that sclero derma is a relatively common but clinically heterogeneous disorder. Th ere are important clinical and prognostic implications in defining lim ited versus diffuse versus overlap disease.