Patterns of interstitial lung disease in primary biliary cholangitis

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To evaluate the occurrence and clinical features of interstitial lung disease (ILD) in a cohort of Russian patients with primary biliary cholangitis (PBC).

Material and methods

PBC patients without concomitant rheumatic diseases were enrolled in our prospective cohort study. All patients underwent chest high-resolution computed tomography (HRCT). The studied outcomes were ILD-related death and liver transplantation or death from liver cirrhosis complications.


ILD was detected in 38 (40.9%) of 93 patients. HRCT patterns of ILD-PBC included sarcoid-like changes, subclinical ILD, organizing pneumonia, unclassifiable interstitial pneumonia, non-specific interstitial pneumonia, and usual interstitial pneumonia. In a multivariate analysis, the absence of liver disease symptoms at the disease presentation, the presence of hepatic non-necrotizing epithelioid cell granulomas, higher serum IgM, and higher blood leukocyte count were associated with ILD. Fourteen (36,8%) patients with ILD showed no respiratory symptoms and only one patient died from complications of ILD. Patients with ILD had better liverrelated survival.


The results of our study showed that ILD is a common finding in PBC. PBC-associated ILD had a benign course and was associated with a lower liver disease severity. Interstitial lung involvement in PBC should be included in a list of differential diagnosis of ILD.

Key words

Interstitial lung disease, primary biliary cholangitis, sarcoid-like pattern, liver transplant-free survival.