Clinical and Demographic Features of Primary Biliary Cholangitis in Kazakhstan

TL;DR

This study describes the clinical features and treatment outcomes of primary biliary cholangitis in Kazakhstan, highlighting late diagnosis and high cirrhosis rates.

Contribution

The first comprehensive study on PBC in Kazakhstan, revealing unique clinical and demographic patterns in Central Asia.

Findings

50.2% of PBC patients in Kazakhstan had cirrhosis at diagnosis.

56.1% of patients showed autoimmune hepatitis features, linked to higher cirrhosis and portal hypertension rates.

Approximately 55% of patients achieved biochemical response to UDCA treatment within one year.

Abstract

Background/Objectives: Primary biliary cholangitis (PBC) is a chronic immune-mediated cholestatic liver disease with increasing global prevalence. However, data on this disease from Central Asia are lacking. We aimed to describe the clinical, serological, and treatment characteristics of PBC patients in Kazakhstan. Methods: This study was a multicenter, retrospective, observational study including adults diagnosed with PBC between 2014 and 2022 across seven hepatology centers in Kazakhstan. Clinical presentation, laboratory parameters, autoimmune comorbidities, liver disease severity, and ursodeoxycholic acid (UDCA) treatment response were assessed. Biochemical response at 1 year was evaluated using Paris-1 and Barcelona criteria. Results: A total of 230 patients were included; 93.9% were female and 91.3% were of Asian ethnicity, with a median age at diagnosis of 53 years. Cirrhosis was present at diagnosis in 50.2% of the patients. PBC with autoimmune hepatitis (AIH) features was identified in 56.1% of the patients and was associated with higher rates of cirrhosis, portal hypertension complications, antinuclear antibody (ANA) positivity, and higher elastography indices compared with isolated PBC. Overall, approximately 55% of the patients achieved a biochemical response to UDCA at 1 year, with similar response rates between patients with PBC and those with PBC with AIH features. Conclusions: This first comprehensive study of PBC in Kazakhstan demonstrates late disease presentation with a high burden of cirrhosis and frequent AIH features. Despite advanced disease, about half of the patients achieved biochemical remission on UDCA. These findings underscore the need for earlier diagnosis and optimized management strategies for PBC in Kazakhstan and similar settings in Central Asia.