Lung involvement in primary biliary cholangitis (PBC)

Background: A subgroup of patients with PBC has concomitant lung involvement. Occasionally lung involvement (LI) occurs first and/or predominates in the clinical course. The objective of our trial was to assess association of LI with other clinical and laboratory signs in PBC. Methods: 68 patients with PBC divided into 2 groups (with and without LI) were observed in Tareev clinic (Sechenov University) in 2018-2020. Diagnosis, age, gender, clinical and laboratory signs have been compared in both groups. Results: Computed tomography revealed LI in 36 (52,9%) cases: 27 – interstitial lung disease (ILD), 4 – multiple nodular lesions, 3 – combined interstitial and nodular lesions and 2 – bronchiolitis. Intrathoracic lymphadenopathy was observed in 15 (22.1%) patients. Among all patients with ILD, 6 (8.8%) had CT-pattern of organizing pneumonia, 1 (1.5%) – non-specific interstitial pneumonia, 1 (1.5%) – usual interstitial pneumonia, 9 (13.2%) – undifferentiated interstitial pneumonia and 9 (13.2%) – sarcoid-like pattern of ILD. Such laboratory tests as ESR (41.3±19.1 and 30.3±20.2 mm/h with and without LI; p = 0.025), IgM level (3.80±2.53 and 2.49±2.27 g/l with and without LI; p Conclusion: The results indicate the association between the presence of LI and some laboratory features in patients with PBC. Some patients may have pulmonary onset that must be taken into account in the diagnosis of this disease.