Abstract
To analyze the clinical features and prognosis of patients with primary biliary cirrhosis (PBC) complicated with interstitial lung disease (ILD). 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. 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. 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.
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