原发性胆汁性肝硬化的临床特点

目的：探讨原发性胆汁性肝硬化(primary biliary cirrhosis，PBC)患者的临床特征，为临床诊断提供思路。方法：搜集2013年1月至2015年1月中南大学湘雅二医院收治的102例原发性胆汁性肝硬化患者的临床表现、生化检查、自身抗体、肝组织病理检查结果进行回顾性分析。结果：102例PBC患者中91例(89.21%)为女性，临床表现主要为乏力、纳差、口干、厌油、恶心、呕吐、皮肤瘙痒、腹痛、腹胀、消化道出血；体征主要表现为黄疸、脾大、肝大、双下肢浮肿、腹水；生化检查主要表现为碱性磷酸酶(alkaline phosphatase，ALP)及γ谷氨酰转肽酶(gamma glutamyl transpeptidase，GGT)升高，GGT升高较突出。抗线粒体抗体M2亚型(anti-mitochondrial antibodies-M2，AMA-M2)阳性组(81例)与AMA-M2抗体阴性组(21例)比较生化指标差异无统计学意义(P>0.05)。肝硬化组(46例)与无肝硬化组(56例)中两组血清抗核抗体(anti-nuclear antibodies，ANA)、抗线粒体抗体M2亚型(anti-mitochondrial antibodies-M2，AMA-M2)、抗核膜糖蛋白抗体(anti-gp210 antibodies，抗gp210)、抗可溶性酸性磷酸化核蛋白抗体(anti-Sp 100 antibodies，抗Sp100)、抗-Ro52抗体(anti-Ro52 antibodies，抗Ro52)、抗前髓白血病细胞抗体(anti- promyelocytic leukemia antibodies，抗PML)阳性率分别为54.35%，89.13%，41.30%，13.04%，43.38%，10.87%和57.14%，71.43%，42.86%，12.5%，51.79%，3.71%，两组比较差异无统计学意义(P>0.05)；而抗三联体抗体(anti-BCOADC-E2 PDC-E2 OGDC-E2 antibodies，抗3E-BPO)在肝硬化组与无肝硬化组阳性率分别为84.78%和58.93%，差异有统计学意义(P<0.05)。30例肝组织病理检查确诊PBC患者的AMA-M2抗3E-BPO阳性率比其他抗体的阳性率高。结论：PBC好发于中年女性，临床表现多样；AMA-A2与抗3E-BPO串联检测有助于提高诊断的阳性率；抗3E-BPO抗体可能作为评价PBC严重性的指标；对于自身抗体阴性的疑似患者，需进一步做肝组织病理检查以确诊。

Clinical features of patients with primary biliary cirrhosis

Objective: To determine features of the clinical manifestation in patients with primary biliary cirrhosis (PBC), and to provide a scientific basis for diagnosis of PBC. Methods: A total of 102 patients with PBC treated in the Second Xiangya Hospital, Central South University, from January 2013 to January 2015 were retrospectively analyzed. The patients’ general condition, clinical manifestations, serum biochemical and immunological parameters were detected. Results: Of the 102 PBC patients, 91 (89.21%) patients were female. The main symptoms in these patients were fatigue, poor appetite, dry mouth, nausea, vomiting, pruritus, stomachache, and abdominal distension. The major signs were jaundice, splenomegaly, hepatomegaly, edema, and ascites. The main features of serum biochemical parameters in these patients included the increase of alkaline phosphatase and gamma glutamyltranspeptidase (GGT), especially the GGT. The anti-mitochondrial antibodies-M2 (AMA-M2) in 81 and 21 patients was positive and negative, respectively. The differences between the AMA-MA positive and negative groups were not statistically significant (P>0.05). According to clinical manifestation, 102 patients were classified into 2 groups: A non-cirrhosis group (n=56) and a cirrhosis group (n=46). The positive rates in these 2 groups, such as ANA, AMA-M2, anti-gp210, anti-Sp100, anti-Ro52, anti-PML, were 54.35%, 89.13%, 41.30%, 13.04%, 43.38% and 10.87% vs 57.14%, 71.43%, 42.86%, 12.5%, 51.79% and 3.71%, respectively, with no significant difference between them (P>0.05). However, there was significant difference in the positive rate of anti-3E-EPO between the above 2 groups (86.78% vs 58.93%, P<0.05). The positive rates of AMA-M2 and anti-3E-EPO in 30 patients diagnosed by hepatic histopathological examination were higher than those of other antibodies. Conclusion: PBC mainly affects middle-aged women, and its clinical manifestation is various. The autoantibody tests play an important role in diagnosis of PBC. Checking for AMA-A2 and anti-3E-BPO can improve the positive rate of PBC. Liver histopathological examination may provide useful information on disease severity, which can determine the histological stage when the patient’s serum autoantibodies are negative.