原发性胆汁性胆管炎并发或不并发自身免疫性疾病患者的临床特点及预后分析

Clinical characteristics and prognosis of patients with primary biliary cholangitis complicated with or without autoimmune diseases

目的评估原发性胆汁性胆管炎(PBC)、PBC并发其他自身免疫性疾病(ATD)患者的临床特点及预后。方法纳入2008年9月至2018年12月收治的PBC患者152例。比较单纯PBC、PBC并发AIH(PBC-AIH)、PBC并发SS(PBC-SS)临床资料,分析预后。结果152例PBC中,单纯PBC 104例(68.4%)、PBC-AIH 11例(7.2%)、PBC-SS 24例(15.8%)、类风湿性关节炎3例(2.0%)、系统性红斑狼疮、Graves病、AIH+SS各2例(1.3%)及SS+类风湿性关节炎、SS+系统性红斑狼疮、SS+Graves病、AIH+SS+类风湿性关节炎各1例(0.6%)。单纯PBC、PBC-AIH患者PT为12.0(11.1,13.9)s、12.8(11.7,17.3)s,差异有统计学意义(P<0.05)。单纯PBC、PBC-AIH患者ANA阳性为55例(52.9%)、9例(81.8%),差异有统计学意义(P<0.05);单纯PBC患者WBC、Hb、Alb及HDL为4.5(3.4,6.2)×109/L、114.2(106.1,128.4)g/L、34.6(29.8,38.7)g/L及1.1(1.0,1.5)mmol/L,PBC-SS患者为3.2(2.6,4.2)×109/L、111.2(79.8,117.0)g/L、33.3(28.2,36.5)g/L及0.9(0.5,1.2)mmol/L,差异有统计学意义(P<0.05)。单纯PBC、PBC-AIH及PBC-SS患者中位生存时间分别为72.0、58.5和50.0个月(P<0.05)。单纯PBC患者5年生存率(80.8%)高于PBC-AIH(7/11例)、PBC-SS患者(58.3%)(P<0.05)。结论AIH是PBC患者肝脏共病中最常见的,而SS是最常见的肝外合并症,PBC并发ATD将影响患者临床预后。

Objective To evaluate the clinical characteristics and prognostic factors in patients with primary biliary cholangitis(PBC),focusing on those with concurrent autoimmune diseases(ATD)in the PBC patient population.Methods A retrospective analysis was conducted on 152 PBC patients,who were admitted between September 2008 and December 2018.The cohort consisted of 17 males and 135 females,aged 52(41,64)years.All patients met the diagnositic criterria for PBC and received treatment with ursodeoxycholic acid(UDCA).Clinical data and prognostic outcomes between PBC patients without concurrent autoimmune diseases and those with concurrent autoimmune hepatitis(PBC-AIH)or Sjogren Syndrome(PBC-SS)were compared.Results Out of 152 evaluated cases of PBC,104 cases were identified as isolated PBC(68.4%),11 as PBC-AIH(7.2%),and 24 as PBC-SS(15.8%).Additionally,3 patients were diagnosed with rheumatoid arthritis(2.0%),2 with systemic lupus erythematosus,2 with Graves’disease and 2 with coexisting AIH and SS(1.3%).In comparing isolated PBC with PBC-AIH,the PT was significantly different at 12.0(11.1,13.9)s and 12.8(11.7,17.3)s,respectively(P<0.05)Similarly,ANA(+)was significantly different between isolated PBC and PBC-AIH,with 55 cases(52.9%)and 9 cases(81.8%),respectively(P<0.05).Furthermore,comparisons of WBC,Hb,Alb and HDL levels were made between isolated PBC and PBC-SS groups.In the isolated PBC group,these values were 4.5(3.4,6.2)×10^(9)/L,114.2(106.1,128.4)g/L,34.6(29.8,38.7)g/L and 1.1(1.0,1.5)mmol/L.17.0)g/L,respectively.In the PBS-SS group,these values were 3.2(2.6,4.2)×10^(9)/L,111.2(79.8,117.0)g/L,33.3(28.2,36.5)g/L and 0.9(0.5,1.2)mmol/L]respectively,revealing statistically significant differences(P<0.05).Survival data were collected up to January 1st,2022.The median survival times for patients with isolated PBC,PBC-AIH and PBC-SS were 72.0,58.5 and 50.0 months,respectively.The median survival time of patients with isolated PBC was significantly longer than those with PBC-AIH and PBC-SS(P<0.05).Similarly,the 5-year survival rate for patients with isolated PBC(80.8%)was significantly higher than for patients with(63.6%)and PBC-SS(58.3%)(P<0.05).Conclusion AIH emerges as the most prevalent hepatic complication among PBC patients,whereas SS stands out as the dominant extrahepatic complication.When PBC becomes complicated with ATD,it poses a detrimental impact on patients’clinical prognosis.