原发性胆汁性胆管炎临床特征及红细胞分布宽度与血小板计数比值对肝硬化的诊断价值分析

  目的  探究原发性胆汁性胆管炎(primary biliary cholangitis，PBC)的临床特征及红细胞分布宽度与血小板计数比值(RDW-to-platelet ratio, RPR)对PBC肝硬化期的诊断价值，提高临床医生对该病的诊疗水平。  方法  收集2014年1月—2019年12月蚌埠医学院第一附属医院感染科门诊及住院部初次确诊为PBC的患者61例，对入组患者的临床资料进行回顾性分析。依据肝硬化诊断标准分为肝硬化组和非肝硬化组，比较2组的血常规、生化指标、RPR值、免疫球蛋白及补体水平。通过受试者工作特征曲线下面积(AUC)判断RPR对PBC肝硬化期的诊断价值。  结果  本组61例PBC患者中女性47例(77.05%)；临床主要表现为黄疸(44.26%)和腹胀(24.59%)；干燥综合征是最常合并的肝外自身免疫疾病；56例(91.80%)出现肝功能异常，以谷氨酰转肽酶(GGT)升高为主；抗线粒体抗体(anti-mitochondrial antibody, AMA)和(或)AMA-M2阳性率为93.44%；肝硬化组和非肝硬化组红细胞分布宽度(RDW)、血小板计数(PLT)、RPR及补体C3、C4比较差异均有统计学意义(均P < 0.05)。RPR诊断肝硬化的AUC值为0.705(95% CI:0.567~0.843；P=0.008), 最佳界值0.471, 灵敏度和特异度分别为52.20%和81.60%。  结论  PBC好发于中年女性，黄疸是最常见的临床表现。碱性磷酸酶(ALP)和GGT的升高以及AMA阳性是该病的主要特点。干燥综合征是最常合并的肝外自身免疫疾病。RPR对PBC肝硬化诊断有较高的特异度，可结合其他无创血清学指标协助评估疾病进程。 

Clinical characteristics of primary biliary cholangitis and RPR value in diagnosing cirrhosis

  Objective  To investigate the clinical features of primary biliary cholangitis (PBC) and the value of red blood cell distribution width-to-platelet ratio (RPR) in diagnosing PBC progression to cirrhosis, as well as to improve the level of diagnosis and treatment of the disease by clinicians.  Methods  From January 2014 to December 2019, 61 patients were diagnosed as PBC in the Outpatient and Inpatient Department of the First Affiliated Hospital of Bengbu Medical College. The clinical data of the patients were retrospectively analysed. According to the diagnostic criteria of cirrhosis, it was divided into cirrhosis and non-cirrhosis groups. Blood routine, biochemical indices, RPR values, immunoglobulin, and complement levels were compared between the two groups. The diagnostic value of RPR for PBC cirrhosis was judged by the area under the working characteristic curve (AUC).  Results  Amongst the 61 patients with PBC, 47 were female (77.05%). The main clinical manifestations were jaundice (44.26%) and abdominal distension (24.59%). Sjogren's syndrome was the most common extrahepatic autoimmune disease. A total of 56 cases (91.80%) had abnormal liver function, primarily GGT increase. The positive rate of anti-mitochondrial antibody (AMA) and/or AMA-M2 was 93.44%. Significant differences were observed in RDW, PLT, RPR, and complement C3 and C4 between cirrhotic and non-cirrhotic groups. The AUC value of RPR in diagnosing liver cirrhosis was 0.705 (95% CI: 0.567-0.843; P=0.008). The best value was 0.471, and the sensitivity and specificity were 52.20% and 81.60%, respectively.  Conclusion  PBC usually occurs in middle-aged women, and jaundice is the most common clinical manifestation. The increase in ALP and GGT and the positive AMA are the main characteristics of the disease. Sjogren's syndrome is the most commonly associated extrahepatic autoimmune disease. RPR has a high specificity in diagnosing PBC progression to cirrhosis and can be combined with other non-invasive serological indicators to help evaluate the disease progress.