Abstract

Primary biliary cholangitis (PBC) is a progressive autoimmune cholestatic liver disease that primarily affects females more than males and may lead to end-stage liver disease. We studied baseline characteristics, and the biochemical and clinical response to ursodeoxycholic acid (UDCA), a first-line treatment option for patients with PBC, in a cohort of patients diagnosed with PBC from Saudi Arabia. A total of 30 patients meeting the diagnostic criteria of PBC who were being followed in the King Faisal Specialist Hospital and Research Centre in Riyadh, Saudi Arabia, between January 1, 2008, and December 31, 2017, were enrolled in the study. The patients with autoimmune hepatitis, primary sclerosing cholangitis, and overlap syndromes were excluded. The baseline characteristics of the patients were recorded. The response to UDCA treatment was assessed according to the Barcelona, Paris I, Paris II, and Toronto criteria, and clinical outcomes, and biochemical changes were ascertained. The mean age was 46 years ± 11.7 with a female gender predominance of 93% (n = 28). Pruritus was the predominant symptom reported by 90% (n = 27) of the patients. A total of 23 (77.7%) patients underwent liver biopsy and all of them showed histological features suggestive of PBC. Cirrhosis was documented in 30% (n = 9) of the patients at baseline. Overall, 86.7% (n = 26) of the patients were positive for antimitochondrial antibodies. The biochemical response rates to UDCA based on Paris I, Paris II, Barcelona, and Toronto criteria were 73.3, 40, 56.7, and 53.3%, respectively. Over 10 years, 30 patients with PBC were diagnosed at a large referral center in Saudi Arabia. The response to UDCA treatment was inadequate.

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