Primary Biliary Cholangitis in Men: Clinical Features, Disease Severity, and Management

Abstract

Background: Primary biliary cholangitis is a chronic autoimmune cholestatic liver disease that predominantly affects women; however, male patients may present with delayed recognition and more advanced disease. Data on primary biliary cholangitis in men remain limited in North African settings. This study aimed to describe the clinical, biochemical, immunological, histological, therapeutic, and prognostic characteristics of male patients with primary biliary cholangitis managed at a tertiary care centre in Morocco. Methods: This retrospective descriptive single-centre study included 40 patients diagnosed with primary biliary cholangitis between January 2021 and December 2025. Six male patients were identified and analysed. Diagnosis was based on chronic cholestatic liver test abnormalities, primary biliary cholangitis-specific autoantibodies when available, compatible liver histology in selected cases, and exclusion of extrahepatic biliary obstruction or primary sclerosing cholangitis. Clinical presentation, biochemical and immunological findings, histological stage, treatment, biochemical response, and prognostic scores were reviewed. Quantitative variables were summarised as medians and ranges, and qualitative variables as numbers and percentages. Results: Male patients represented 15.0% of the cohort, with a female-to-male ratio of approximately 5.7:1. The median age at diagnosis was 47.5 years (range: 20–65 years). Pruritus was present in five patients, asthenia in four, and jaundice in three. Cirrhosis and signs of portal hypertension were documented at diagnosis in three patients. Antimitochondrial antibodies were positive in four patients; one antimitochondrial antibody-negative patient had anti-sp100 positivity, and one required histological confirmation. Liver biopsy was performed in four patients, and advanced Ludwig stage III–IV disease was found in three. All patients received ursodeoxycholic acid. Among four patients evaluable at 12 months, two achieved a complete biochemical response, one had a partial response, and one had no response. Conclusion: In this small retrospective male subgroup, primary biliary cholangitis was frequently symptomatic and advanced at diagnosis. These findings support early recognition of unexplained cholestasis in men, prompt treatment with ursodeoxycholic acid, and long-term surveillance for disease progression and hepatocellular carcinoma, particularly in patients with cirrhosis. Given the small sample size, these observations should be regarded as hypothesis-generating and confirmed in larger prospective multicentre studies incorporating direct male-versus-female comparisons.