目的 研究原发性胆汁性肝硬化(PBC)的临床病理特点、伴发疾病及治疗反应，以提高对PBC的认识。方法 分析37例PBC的临床资料及其中13例肝穿刺病理资料：抗线粒体抗体(AMA)采用间接免疫荧光法检测，M2亚型采用ELISA法检测。结果 ① 37例患者，发病年龄(50±10)岁。②症状：乏力20例(54.1%)，瘙痒10例(27.0%)，腹痛8例(21.6%)，肝性脑病1例(2.7%)；体征：肝大26例(70.3%)，脾大20例(54.1%)，黄疸16例(43.2%)，腹水4例(10.8%)，门脉高压症7例(18.9%)。③实验室检查：血清碱性磷酸酶水平升高37例(100.0%)，(450±322) U/L；总胆红素升高31例(83.8%)，(126±14) μmol/L；丙氨酸转氨酶升高32例(86.5%)，(71±17) U/L；IgM升高32例(86.5%)；ANA阳性10例(27.0%)；抗SSA和/或抗SSB抗体阳性5例(13.5%)。④合并干燥综合征(SS)：符合哥本哈根标准17例(45.9%)，符合董怡标准10例(27.0%)。⑤肝穿刺病理：取材满意9例中，胆管炎9例，肝细胞点状及碎片状坏死1例，纤维化8例。⑥治疗反应：单纯皮质激素和/或免疫抑制剂治疗者26例，有效10例，无效16例，有效组IgG升高及合并SS例数均显著高于无效组(P分别＜0.01和0.05)。结论 PBC中年女性多见，以肝脾大和黄疸、乏力为主要临床表现，容易合并SS。肝功能异常以胆汁淤积为主，病理主要表现为非化脓性胆管炎而肝细胞病变相对较轻。皮质激素和/或免疫抑制剂治疗对PBC多无效，只适用于合并SS或IgG升高的PBC。%Objective To investigate clinical and pathological features of primary biliary cirrhosis (PBC),its association with Sjgren′s syndrome (SS) and response to therapy so as to improve management of this disorder.Methods Clinical data of 37 PBC patients together with 13 liver biopsy specimens were reviewed.Antimitochondrial antibodies (AMA) were detected by using indirect immunofluorescent method and M2 subtype by ELISA.Results ① Mean age at disease onset was (50±10) years;② Symptoms:fatigue 20(54.1%),pruritus 10(27.0%),abdominal discomfort 8 (21.6%),hepatic encephalopathy 1(2.7%);Signs:hepatomegaly 26(70.3%),splenomegaly 20(54.1%),jaundice 16(43.2%),ascites 4(10.8%),portal hypertension 7(18.9%)；③ Laboratory tests:serum alkaline phosphatase elevation 37(100.0%),(450±322) U/L;Total bilirubin elevation 31 (83.8%),(126±14) μmol/L;serum alanine aminotransferase elevation 32(86.5%),(71±17) U/L;IgM elevation 32(86.5%);In 10(27.0%) antinuclear antibodies were positive and in 5(13.5%) anti-SSA and/or anti-SSB were positive;④ SS association:17(45.9%) fulfilled Copenhagen criteria,Ten (27.0%) fulfilled Dongy′s criteria;⑤ Liver biopsy:among 9 satisfactorily sampled specimens,all had cholangitis,1 had piecemeal necrosis and 8 had fibrosis;⑥ Treatment response:26 patients received corticosteroid and/or immunosuppressive agent and only 10 responded well while 16 failed.IgG elevation and SS in the effective group were more common than in the failed group (P＜0.01 and＜0.05 respectively).Conclusion PBC affects middle-aged women with hepatomegaly,splenomegaly jaundice and fatigue as presenting features and is closely associated with SS.Liver function tests typically reveal a cholestatic pattern.The characteristic pathological features of PBC are non-suppurative cholangitis,while hepatocyte involvement is relatively mild.PBC does not respond well to corticosteroid.Only for those PBC associated with IgG elevation and SS,should corticosteroid be considered.
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