To investigate the incidence and prevalence, as well as the mortality and survival rates, of systemic sclerosis (SSc) in a defined area of northwest Greece with a population of about 500,000 inhabitants.
Cases have been recorded from the following sources: (1) inpatients and outpatients referred to the Rheumatology Clinics of the Ioannina University Hospital and the Ioannina General Hospital; (2) patients referred to the private rheumatologists practicing in the study area. All patients recorded between 1/1/1981 and 31/12/2002, resident in the study area, were included in the study. Diagnosis was based on the American College of Rheumatology classification criteria for SSc. Incidence and prevalence rates were calculated as number of cases per 10(5) inhabitants. Population data were based on the National Census of 1981, 1991, and 2001.
The age-adjusted prevalence of SSc was 15.40 cases/10(5) inhabitants on 31/12/2002. A total of 109 new cases were diagnosed during the study period, giving a mean annual age-adjusted incidence rate of 1.10 cases/10 5 inhabitants. There were 98 women and 11 men, giving a ratio of 8.9/1. Limited SSc was diagnosed in 75% and diffuse in 25% of the patients. Esophageal involvement was found in 59%, lung involvement in 56%, and renal disease in 5%. Thirty-six deaths were recorded during the study period in this incidence cohort. The 5-year survival rate was 83% and the 10-year survival rate was 70%.
The incidence and prevalence of SSc in northwest Greece were found to be lower than those of the USA and Australia, and higher than those of northern European countries and Japan. The survival rates were similar to those reported by other studies.