Primary biliary cirrhosis complicated with interstitial lung disease: a prospective study in 178 patients.

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.