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Advances in Respiratory Medicine is published by MDPI from Volume 90 Issue 4 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with Via Medica.
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Case Report

Interstitial Lung Disease in Patients with Primary Biliary Cirrhosis

by
Małgorzata Bartosiewicz
1,
Izabela Siemion-Szcześniak
1,
Małgorzata Jędrych
1,
Piotr Radwan-Röhrenschef
1,
Katarzyna Lewandowska
1,
Renata Langfort
2,
Karina Oniszh
3,
Monika Franczuk
4 and
Jan Kuś
1,*
1
Department of Pulmonary Diseases, National Tuberculosis and Lung Diseases Research Institute, ul. Płocka 26, 01-138 Warszawa, Poland
2
Department of Pathomorphology, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warszawa, Poland
3
Department of Radiology, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warszawa, Poland
4
Department of Respiratory Pathophysiology, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warszawa, Poland
*
Author to whom correspondence should be addressed.
Adv. Respir. Med. 2012, 80(5), 471-481; https://doi.org/10.5603/ARM.27559
Submission received: 28 December 2011 / Revised: 27 August 2012 / Accepted: 27 August 2012 / Published: 27 August 2012

Abstract

Primary biliary cirrhosis (PBC) is a chronic autoimmune disorder of unknown etiology. The disease affects middle-aged women and is characterized by the destruction of the intralobular bile ducts that causes consequent cholestasis. AMA is a hallmark of PBC, composed mostly of IgG and IgM class. The M2 antibody is the most specific one, with sensitivity range of 54–98% depending on type of test used. PBC is often accompanied by other autoimmune diseases, such as Sjøgrens syndrome, thyroiditis, rheumatoid arthritis, dermatomyositis, polymyositis. Interstitial lung disease (ILD) has been reported in patients with primary biliary cirrhosis but its frequency and nature are poorly understood. We report pulmonary involvement in the course of PBC in 4 middle-aged women. Histopatological examination of lung specimens was available in three patients: two presented with sarcoid—like granulomas, one with lymphocytic interstitial pneumonia (LIP). In one patient the diagnosis of pulmonary fibrosis was based on clinical and radiological features. Because of abnormal pulmonary function tests (PFT) results all the patients were treated with prednisone, one, additionally with azathioprine. The treatment was successful in all of the patients.
Keywords: primary biliary cirrhosis; liver; interstitial lung disease; connective tissue disease primary biliary cirrhosis; liver; interstitial lung disease; connective tissue disease

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MDPI and ACS Style

Bartosiewicz, M.; Siemion-Szcześniak, I.; Jędrych, M.; Radwan-Röhrenschef, P.; Lewandowska, K.; Langfort, R.; Oniszh, K.; Franczuk, M.; Kuś, J. Interstitial Lung Disease in Patients with Primary Biliary Cirrhosis. Adv. Respir. Med. 2012, 80, 471-481. https://doi.org/10.5603/ARM.27559

AMA Style

Bartosiewicz M, Siemion-Szcześniak I, Jędrych M, Radwan-Röhrenschef P, Lewandowska K, Langfort R, Oniszh K, Franczuk M, Kuś J. Interstitial Lung Disease in Patients with Primary Biliary Cirrhosis. Advances in Respiratory Medicine. 2012; 80(5):471-481. https://doi.org/10.5603/ARM.27559

Chicago/Turabian Style

Bartosiewicz, Małgorzata, Izabela Siemion-Szcześniak, Małgorzata Jędrych, Piotr Radwan-Röhrenschef, Katarzyna Lewandowska, Renata Langfort, Karina Oniszh, Monika Franczuk, and Jan Kuś. 2012. "Interstitial Lung Disease in Patients with Primary Biliary Cirrhosis" Advances in Respiratory Medicine 80, no. 5: 471-481. https://doi.org/10.5603/ARM.27559

APA Style

Bartosiewicz, M., Siemion-Szcześniak, I., Jędrych, M., Radwan-Röhrenschef, P., Lewandowska, K., Langfort, R., Oniszh, K., Franczuk, M., & Kuś, J. (2012). Interstitial Lung Disease in Patients with Primary Biliary Cirrhosis. Advances in Respiratory Medicine, 80(5), 471-481. https://doi.org/10.5603/ARM.27559

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