[Primary biliary cirrhosis complicated by pulmonary arterial hypertension: a clinical analysis].
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].


CONCLUSIONS
Closely associated with portal hypertension and indicating poor prognosis, moderate to severe PAH is not a rare complication of PBC.
Shen
Min
M
Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhang
Xuan
X
Zhang
Feng-chun
FC
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000276:immunology / Q000628:therapy; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000276:immunology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies
D001323:Autoantibodies