Pulmonary hypertension in primary biliary cirrhosis: A prospective study in 178 patients

Abstract

Objective. To analyze the incidence, clinical features, and prognosis of patients with primary biliary cirrhosis (PBC) complicated by pulmonary hypertension (PH). Material and methods. A total of 178 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 rheumatological examination, laboratory tests (including autoantibodies), and Doppler echocardiography were conducted for each patient and compared between patients with and without PH. Results. Twenty-one PBC patients (11.8%) had PH. Among them, four patients (19.0%) had moderate to severe PH, and one patient died of right heart failure instead of liver failure. The incidences of Raynaud's phenomenon, interstitial lung disease, Sjögren's syndrome, and portal hypertension, the proportion of patients with a positive anti-SSA, the level of serum IgA, as well as the Mayo risk score in the PH–PBC patients were significantly higher than in the non-PH-PBC group (p = 0.02, 0.001, 0.02, 0.03, 0.006, 0.04 and 0.02, respectively). Conclusions. PH, including moderate to severe PH, is not a rare complication of PBC. This complication is closely associated with portal hypertension and immunological dysregulation and indicates a poor prognosis.