171例原发性胆汁性胆管炎10年随访研究

A 10-year follow-up study of 171 cases of primary biliary cholangitis

目的随访原发性胆汁性胆管炎(primary biliary cholangitis,PBC)患者10年病例资料,总结临床特点并进行生存分析,探讨影响PBC的预后相关危险因素。方法于2015年7月1日回顾性收集2008年至2013年首诊于宁夏医科大学总医院及心脑血管病医院门、急诊及住院PBC患者共171例,以此为研究对象并建立档案。自2015年7月后随访患者历次就诊信息及预后,观察结束时间为2023年7月31日。观察患者一般资料、实验室检查结果、相关影像学资料及消化内镜检查结果。采用寿命表法计算生存率,Kaplan-Meier法对影响预后的因素进行单因素分析。将单因素有统计学意义的变量纳入多元Cox回归模型分析。结果171例患者平均年龄(57.71±11.79)岁,男女比例1∶4.9。初次就诊时仅有38.01%患者处于PBC的早期阶段,39.76%合并自身免疫性疾病。PBC患者死亡平均年龄为(61.69±10.96)岁,主要死亡原因为上消化道出血(32.9%)、腹腔感染(23.6%)、重症肺炎(20.7%)。PBC患者平均生存时间64.26个月,1年生存率81.87%、3年生存率为51.46%、5年生存率为39.77%、10年生存率为20.75%。多因素Cox回归分析结果显示延迟诊断(HR 68.035)、熊去氧胆酸应答不佳或失应答(HR 8.081)、血清钙离子(HR 0.629)是影响患者总体生存时间的独立危险因素;延迟诊断(HR 83.302)、乏力(HR 2.668)、联合其他免疫抑制药物(HR 1.733)、血小板<125×109/L(HR 0.997)、体重可以维持(HR 0.189)是影响患者发生并发症的独立危险因素;熊去氧胆酸应答不佳或失应答(HR 4.204)、乏力(HR 1.721)、血小板<125×109/L(HR 0.997)是影响患者影像学进展的独立危险因素。结论PBC多见于中年女性,早期症状缺乏特异性,40%的患者合并其他自身免疫性疾病。消化道出血、腹腔感染、重症肺炎是首要死亡原因。存在延迟诊断、熊去氧胆酸应答不佳或者失应答、血清钙离子≤2.0 mmol/L是影响患者总体生存时间的独立危险因素。

Objective Follow-up data of patients with primary biliary cholangitis(PBC)for 10 years,summarize the clinical characteristics and analyze the survival,and explore the risk factors related to the prognosis of PBC.Methods On July lst,2015,a total of 171 PBC patients who were first diagnosed in the General Hospital of Ningxia Medical University and the Cardiovascular and Cerebrovascular Hospital from 2008 to 2013 were retrospectively collected,and their files were established.After July 2015,the patients'previous medical information and prognosis were followed up,and the observation ended on July 31,2023.Observe the general data of patients,laboratory examination results,relevant imaging data and digestive endoscopy results.The survival rate was calculated by life table method,and the factors affecting prognosis were analyzed by Kaplan-Meier method.The univariate statistically significant variables were included in the multivariate Cox regression model analysis.Results The average age of 171 patients was(57.71±11.79)years,and the ratio of male to female was 1:4.9.At the first visit,only 38.01%of patients were in the early stage of PBC,and 39.76%were complicated with autoimmune diseases.The average death age of PBC patients was(61.69±10.96)years,andthemain causes ofdeath were upper gastrointestinal bleeding(32.9%),abdominal infection(23.6%)and severe pneumonia(20.7%).The average survival time of PBC patients was 64.26 months,the 1-year survival rate was 81.87%,the 3-year survival rate was 51.46%,the 5-year survival rate was 39.77%,and the 10-year survival rate was 20.75%.Multivariate COX regression analysis showed that delayed diagnosis(HR 68.035),poor or unresponsive ursodeoxycholic acid(HR 8.081)and serum calcium ion(HR 0.629)were independent risk factors affecting the overall survival time of patients.Delayed diagnosis(HR 83.302),fatigue(HR 2.668),combination with other immunosuppressive drugs(HR 1.733),platelet(HR 0.997)and weight maintenance(HR 0.189)are independent risk factors for complications.Poor or unresponsive ursodeoxycholic acid(HR 4.204),fatigue(HR 1.721)and platelet(HR 0.997)are independent risk factors that affect the imaging progress of patients.Conclusion PBC is more common in middle-aged women,and its early symptoms are not specific.40%patients are complicated with other autoimmune diseases.Gastrointestinal bleeding,abdominal infection and severe pneumonia are the leading causes of death.Delayed diagnosis,poor or unresponsive response of ursodeoxycholic acid and serum calcium ion≤2.Ommol/L are independent risk factors affecting the overall survival time of patients.