Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002

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Objectives

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.

Materials and methods

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 105 inhabitants. Population data were based on the National Census of 1981, 1991, and 2001.

Results

The age-adjusted prevalence of SSc was 15.40 cases/105 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/105 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%.

Conclusions

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.

Section snippets

Materials and methods

The study area represents a population of 488,435 inhabitants according to the National Census of 2001. It is a defined area of northwest Greece including 6 districts, 4 on the mainland and 2 on islands. Urban residents represented 37% of the total population, living in the capitals of the districts. Two rheumatology clinics are implemented in the area, both in Ioannina, the largest city of the study area, with a Medical School and a University Hospital. Eight private rheumatologists are

Results

The main characteristics of SSc patients diagnosed during the period 1981 to 2002 in northwest Greece are presented in Table 1. A total of 109 cases were diagnosed among the population of the area studied. Among them, 104 were recorded at the Rheumatology Clinics and 5 from private rheumatologists. There was a ninefold higher number of women than men, and women had a lower mean age at diagnosis.

Figure 1 shows the incidence of the disease during the study period, by sex and groups of age. The

Discussion

Epidemiological studies performed in several countries suggest significant geographic variations in the occurrence of SSc. According to the results of the present study, SSc incidence and prevalence rates in the area studied are between those of the United States and Australia, and those of Northern European countries and Japan (Table 3) (13, 14, 15, 16, 17, 18, 19, 20, 21, 22). However, the methods used to ascertain cases differ among studies. In the study by Steen and coworkers, cases were

Acknowledgment

This paper is dedicated to Professor H.M. Moutsopoulos for his 60th birthday celebration.

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