Geoepidemiology, clinical manifestations and outcome of primary biliary cholangitis in Greece

Abstract

Background & aims Primary biliary cholangitis (PBC) is a disease with rising prevalence and considerable geographical variation. To describe the prevalence, spatial and time distribution, baseline characteristics, response to treatment, outcome and the validity of GLOBE score in a large cohort of Greek PBC patients as an independent validation of this score has not been done so far. Methods The last 16 years, 482 PBC patients (86.5% females) were evaluated and analysed retrospectively, using a prospectively collected database. Special attention was paid to the assessment of treatment response according to GLOBE score. Results Age at initial evaluation was 56.3 ± 13.7 years. Among 432 Thessaly residents, prevalence was 582/million (non-homogeneous distribution). Nineteen districts showed a prevalence > 800/million. Symptomatic disease onset could be identified in 91 patients, with a significant peak during spring (P = 0.03). At diagnosis, 43.6% were asymptomatic and 16.2% cirrhotic. Male sex (P = 0.02), older age (P < 0.001), alcohol consumption (P < 0.01) and concomitant liver disease (P < 0.001) were negative prognostic factors for cirrhosis. During a median [interquartile range, range] follow-up of 5.1 (7.8, 15.7) years, 62 patients died or underwent liver transplantation. Patients with GLOBE score > 0.30 had significantly worse prognosis (P < 0.001) with 5-, 10-, and 15-year survival rates of 84%, 50% and 42%. Conclusions There is increased PBC prevalence in Thessaly with remarkable geographic clustering and seasonal variability. PBC is diagnosed at early stages although males had a more advanced disease. GLOBE score applies perfectly in Greek patients and this will likely help detecting patients that may benefit from new therapies. © 2017 European Federation of Internal Medicine