open access

Vol 80, No 5 (2012)
CASE REPORTS
Published online: 2012-08-27
Submitted: 2013-02-22
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Interstitial lung disease in patients with primary biliary cirrhosis

Małgorzata Bartosiewicz, Izabela Siemion-Szcześniak, Małgorzata Jędrych, Piotr Radwan-Röhrenschef, Katarzyna Lewandowska, Renata Langfort, Karina Oniszh, Monika Franczuk, Jan Kuś
Pneumonol Alergol Pol 2012;80(5):471-481.

open access

Vol 80, No 5 (2012)
CASE REPORTS
Published online: 2012-08-27
Submitted: 2013-02-22

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 Sjögrens 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.

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 Sjögrens 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.
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Keywords

primary biliary cirrhosis; liver; interstitial lung disease; connective tissue disease

About this article
Title

Interstitial lung disease in patients with primary biliary cirrhosis

Journal

Advances in Respiratory Medicine

Issue

Vol 80, No 5 (2012)

Pages

471-481

Published online

2012-08-27

Bibliographic record

Pneumonol Alergol Pol 2012;80(5):471-481.

Keywords

primary biliary cirrhosis
liver
interstitial lung disease
connective tissue disease

Authors

Małgorzata Bartosiewicz
Izabela Siemion-Szcześniak
Małgorzata Jędrych
Piotr Radwan-Röhrenschef
Katarzyna Lewandowska
Renata Langfort
Karina Oniszh
Monika Franczuk
Jan Kuś

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