Interstitial Lung Disease in Primary Biliary Cholangitis: A Cohort Prospective Study.

Area of Science:

• Pulmonology
• Hepatology
• Immunology

Background:

• Interstitial lung disease (ILD) is an recognized extrahepatic manifestation of primary biliary cholangitis (PBC).
• Limited data exist on the prevalence and clinical significance of ILD in PBC patients.
• Understanding ILD in PBC is crucial for comprehensive patient management.

Purpose of the Study:

• To evaluate the occurrence and clinical features of ILD in a cohort of PBC patients.
• To identify risk factors associated with ILD in PBC.
• To assess the impact of ILD on liver-related and lung-related outcomes in PBC.

Main Methods:

• Prospective cohort study of 93 PBC patients without rheumatic diseases.
• Chest high-resolution computed tomography (HRCT) for ILD detection.
• Assessment of liver-related and lung-related survival outcomes.

Main Results:

• ILD findings were detected in 40.9% of PBC patients via HRCT.
• Sarcoid-like pattern was the most frequent ILD presentation.
• PBC patients with ILD showed less liver cirrhosis, fewer liver symptoms, higher serum IgM, and higher AMA-M2 positivity.
• Independent risk factors for ILD in PBC included absence of liver disease symptoms, hepatic non-necrotizing epithelioid cell granulomas, higher serum IgM, and higher leukocyte count.
• Over a third of ILD patients were asymptomatic; only one ILD-related death occurred.
• Patients with ILD demonstrated better liver transplant-free survival.

Conclusions:

• Interstitial lung disease in primary biliary cholangitis is common and often asymptomatic.
• PBC-associated ILD typically follows a benign clinical course.
• The presence of ILD in PBC is associated with reduced liver disease severity and better transplant-free survival.
• ILD should be considered in the differential diagnosis of patients with PBC.