Objective: To analyze the clinical features and prognosis of patients with primary biliary cirrhosis (PBC) complicated by pulmonary arterial hypertension (PAH).
Methods: The medical records of 80 PBC inpatients, 8 of which were complicated by pulmonary arterial hypertension (PAH) were retrospectively analyzed to compare the differences in clinical features, biochemical parameters, positive rates of autoantibodies and Mayo risk score between the 2 groups with or without PAH.
Results: The prevalence of portal hypertension was 7/8, in the PAH group, significantly higher than that in the non-PAHG group [(44.4%, 32/72), P < 0.05]. The Mayo score of the PAH group was 7.0 +/- 1.2, significantly higher than that in the non-PAH group (5.6 +/- 1.5, P < 0.05). The IgA level of the PAH group was (4.4 +/- 1.9) g/L, significantly higher than that in the non-PAH group [(3.0 +/- 1.8) g/L, P < 0.05]. The serum level of alkaline phophatase of the noon-PAH group was 293 +/- 218 U/L, significantly higher than that of the PAH group [(150 +/- 53) U/L, P < 0.05].
Conclusion: Closely associated with portal hypertension and indicating poor prognosis, moderate to severe PAH is not a rare complication of PBC.