Primary biliary cirrhosis: an Indian experience

Area of Science:

• Hepatology
• Autoimmunology

Background:

• Primary biliary cirrhosis (PBC) is considered a rare liver disease, particularly in India.
• Understanding the prevalence and clinical characteristics of PBC in diverse geographical regions is crucial for accurate diagnosis and management.

Observation:

• A 5-year retrospective analysis was conducted on patients diagnosed with PBC at a tertiary referral center.
• Diagnosis was confirmed using liver biochemistry, histology, and antimitochondrial antibody (AMA) testing, excluding biliary obstruction.

Findings:

• Five cases of PBC were identified, all in female patients.
• Common initial symptoms included pruritus, jaundice, and fatigue. Hepatomegaly was observed in 80% of patients.
• Elevated serum alkaline phosphatase with mild hyperbilirubinemia was characteristic. AMA was positive in 4 out of 5 patients, and 3 out of 4 biopsies indicated advanced disease (Stage III-IV).

Implications:

• The clinical presentation and disease course of PBC in India align with Western cohorts.
• This suggests PBC may be underdiagnosed in India rather than inherently rare.
• Further epidemiological studies are warranted to ascertain the true prevalence of PBC in India.