title: Primary biliary cirrhosis complicated with interstitial lung disease: a prospective study in 178 patients.
abstract: OBJECTIVE
  To analyze the clinical features and prognosis of patients with primary biliary cirrhosis (PBC) complicated with interstitial lung disease (ILD).
  
  
  METHODS
  One hundred and seventy-eight consecutive PBC patients, who were admitted to Peking Union Medical College Hospital from January 2001 to March 2007, were included in this prospective study. A structured interview, systemic rheumatologic examination, laboratory tests (including autoantibodies), and high resolution computed tomography were conducted for each patient and compared between patients with and without ILD.
  
  
  RESULTS
  Twenty-eight (15.7%) PBC patients had ILD, and 53.6% of them had respiratory symptoms including dyspnea and cough, 88.2% showed diffusing and restrictive ventilation impairment on pulmonary function test. PBC patients with ILD were older and had higher erythrocyte sedimentation rate levels than those without ILD, P<0.05. There was no significant difference in liver biochemical parameters, positive rates of antinuclear antibodies, antimitochondrial antibodies (AMA), AMA-M2, anticentromere antibodies, anticardiolipin antibodies, and Mayo risk score between the 2 groups, P>0.05. Logistic regression analysis found that Raynaud phenomenon and association with other connective tissue diseases (CTDs) were risk factors for PBC patients to develop ILD (P=0.04, odds ratio=3.12 and P=0.01, odds ratio=3.18, respectively), though 42.9% of ILD-PBC patients did not have other CTDs.
  
  
  CONCLUSIONS
  ILD is a common and clinically significant complication of PBC. PBC patients who have Raynaud phenomenon and other CTDs are more likely to develop ILD.
authors.affiliation: Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China.
authors.forename: Min
authors.initials: M
authors.lastname: Shen
authors.forename: Fengchun
authors.initials: F
authors.lastname: Zhang
authors.forename: Xuan
authors.initials: X
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D001799:Blood Sedimentation; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D016015:Logistic Models; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011928:Raynaud Disease / Q000150:complications; D012129:Respiratory Function Tests; D012307:Risk Factors; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: D001323:Autoantibodies