title: [Interstitial lung disease in patients with primary biliary cirrhosis].
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.
authors.affiliation: I Klinika Chorób Płuc, Instytut Gruźlicy I Chorób Płuc, ul. Płocka 26, 01–138 Warszawa.
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Bartosiewicz
authors.forename: Izabela
authors.initials: I
authors.lastname: Siemion-Szcześniak
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Jędrych
authors.forename: Piotr
authors.initials: P
authors.lastname: Radwan-Röhrenschef
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Lewandowska
authors.forename: Renata
authors.initials: R
authors.lastname: Langfort
authors.forename: Karina
authors.initials: K
authors.lastname: Oniszh
authors.forename: Monika
authors.initials: M
authors.lastname: Franczuk
authors.forename: Jan
authors.initials: J
authors.lastname: Kuś
mesh_terms: D002756:Cholagogues and Choleretics / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000000981:diagnostic imaging* / Q000188:drug therapy; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000000981:diagnostic imaging*; D008875:Middle Aged; D011044:Poland; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed; D014580:Ursodeoxycholic Acid / Q000008:administration & dosage*
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid