SYSTEMIC-SCLEROSIS IN ICELAND - A NATIONWIDE EPIDEMIOLOGIC-STUDY

Objectives-To investigate the incidence, prevalence and clinical featu res of systemic sclerosis (SS) in Iceland. Methods-AU patients diagnos ed with SS from 1975-90 were included. Retrieval for the study began i n 1980 and was carried out by computerised search from registers of al l hospitals and health care clinics and death registration files, and with personal communication with doctors in Iceland Results-Over a 16 year period from 1975-90, 15 new cases were found with an incidence of 0.7 and 0.05/100 000, for females and males at risk respectively, and 0.38 for both sexes. At the end of 1990 there were 18 patients alive with SS, 13 with limited and five with diffuse cutaneous involvement. The standardised prevalence was 11.9 1.5/100 000 for females and males at risk respectively. The crude prevalence rate for both sexes was 7. 1/100 000. There were five deaths, two patients died of SS related cau ses, one had SS renal disease. The relative risk of death was similar to that in the general population. The calculated five year survival r ate was 100% and the 10 year survival rate 81%. No HLA antigen associa tion was found. Conclusion-Compared with previous surveys this study s hows a low incidence of systemic sclerosis and a high proportion of pa tients with limited cutaneous involvement.