Introduction: Progressive systemic sclerosis (PSS) is an inflammatory disease of connective tissue, with onset as edema that continues with fibrosis, induration, and skin atrophy, followed by attacks on the joints, internal organs, and secondary proliferation of connective tissue. Purpose: To research in which residence locations and among which group age is the most frequent incidence, prevalence and clinical manifestations of systemic sclerosis in Dukagjini Plain which is inhabited by 698450 resident citizens. Material and methods: 51 patients with progressive systemic sclerosis were studied, out them 44 were females (86.3%) and 7 males (13.7%) respectively, during the period from 2005 to 2010. Their illness was active from 18 to 60 months in accordance with EUSTAR criteria. They are of different age, median age is 44.2 ±10.1. Their diagnose is determined based on revised ACR criteria. Prevalence of patients with PSS was 14.61/100.000, while the incidence was 2.8/100.000, whereas CI (Confidence interval) or limit of accuracy was 95%. Results: Largest number of patients per 100.000 citizens has Istog municipality which has the largest number of patients with PSS. It is followed by Mamusha and Rahovec municipalities. The largest examined group age is 35-44 year old, 41.2% respectively. Conclusion: additional studies are necessary to carry out in order to find the reasons of asymmetrical distribution of patients with systemic sclerosis in the municipalities of Dukagjini Plain.
Key words: systemic sclerosis, incidence, prevalence, Dukagjini Plain, clinical features
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