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目的对原发性胆汁性肝硬化(PBC)合并肺动脉高压(PAH)患者的临床和预后进行分析,提高临床对PBC的诊治水平。方法回顾性分析80例PBC患者(其中8例合并PAH)的临床表现、抗体谱、生化指标和预后指标(Mayo危险评分),并对合并PAH及不合并PAH的PBC患者进行了上述指标的比较。结果门脉高压症的发生比例(率):PAH组为7/8,无PAH组为44.4%(32/72),两组比较P<0.05;Mayo危险评分和IgA水平PAH组亦高于无PAH组(P<0.05)。γ谷氨酰转移酶(GGT)和碱性磷酸梅(ALP)的平均值无PAH组高于PAH组,两组间的ALP差异有统计学意义(均P<0.05)。结论PBC患者可以合并PAH,其中中重度PAH并不少见。PBC合并PAH与门脉高压症相关,其出现提示预后不良。临床医师需提高对PBC和PAH的认识,早期治疗,改善预后。
Objective To analyze the clinical and prognosis of patients with primary biliary cirrhosis (PBC) complicated with pulmonary hypertension (PAH) and to improve the clinical diagnosis and treatment of PBC. Methods The clinical manifestations, antibody profiling, biochemical and prognostic factors (Mayo risk score) were analyzed retrospectively in 80 patients with PBC (8 patients with PAH). The above indexes were compared between PBC patients with and without PAH . Results The incidence rate of portal hypertension was 7/8 in PAH group and 44.4% (32/72) in non-PAH group, P <0.05 in both groups; Mayo risk score and IgA level in PAH group were also higher than those in non-PAH group PAH group (P <0.05). The mean values of GGT and ALP in PAH group were higher than those in PAH group, and the differences of ALP between the two groups were statistically significant (P <0.05). Conclusion Patients with PBC can be combined PAH, including moderate to severe PAH is not uncommon. PBC combined PAH and portal hypertension, the emergence of poor prognosis prompted. Clinicians need to raise awareness of PBC and PAH, early treatment, and improve prognosis.