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Abstract
Background and Objective:Primary biliary cholangitis is a rare disease with scarce epidemiological data. The aim of this study was to characterize a cohort of patients in Portugal and to evaluate the response to treatment and development of complications.
Methods:This retrospective observational study included patients with diagnostic criteria of primary biliary cholangitis from a single center. Data on disease presentation, laboratory results, treatment and clinical endpoints were collected and analyzed. Statistical significance was established at p<0.05.
Results:Fifty-three patients were included, 89% were women, with mean age of 62 ± 15 years at diagnosis. The majority were asymptomatic (49%), tested positive for AMA (96%), had increased alkaline phosphatase (median= 214 U/L) and were in Ludwig’s stage I (42%). Overlap syndrome was diagnosed in seven patients (13%) and nine patients (17%) bared overlap features without assenting biopsy. All patients were treated with ursodeoxycholic acid and 56% achieved biochemical response at one year. Patients with overlap features and higher alkaline phosphatase presented a significantly greater decrease in alkaline phosphatase levels.
Conclusions:The baseline characteristics of this cohort were in agreement with those previously described. Outstandingly, 30% of patients had features of overlap syndrome and presented a steeper reduction in alkaline phosphatase.
