■自身免疫性肝病(AILD)包括自身免疫性肝炎(AIH),原发性胆汁性胆管炎(PBC)和原发性硬化性胆管炎(PSC)三者之间存在重叠。我们分析了到印度三级护理中心就诊的AILD患者的频谱和治疗结果。
■对2008年6月至2021年4月的AILD患者进行回顾性分析。诊断是基于临床,生物化学,成像,血清学,和组织学特征。符合条件的患者根据疾病阶段接受治疗。对治疗的生化反应定义为AST的正常化,ALT,胆红素,AIH6个月时的免疫球蛋白G水平,PBC1年总胆红素和/或白蛋白正常化,PSC碱性磷酸酶(ALP)水平下降40%。
■分析了二百七十五名患者。AIH(58.54%)最常见,其次是AIH-PBC(24%)和AIH-PSC(6.54%)的重叠,PSC(6.18%),和PBC(4.72%)。大多数病人出现在第三或第四个十年,除了主要发生在第5个十年的PBC。大多数患者为女性(72.72%)。黄疸是60%患者中最常见的表现。57.47%的患者出现肝硬化。重叠患者有更多的瘙痒(54.76vs6.83%),疲劳(63.1%对49.7%),肝肿大(52.4%vs25.5%),与单独AIH患者相比,ALP更高(80.9%vs37.7%)。33例患者(13.5%)出现急性表现,大多数患有AIH发作。5例患者患有急性肝衰竭(ALF),9例患有慢性急性肝衰竭(ACLF)。ALF与80%的死亡率相关,而55.56%的ACLF患者对免疫抑制有完全的生化反应。在接受免疫抑制的AIH和/或重叠患者中,60.69%的患者对免疫抑制有完全的生化反应.高ALT(OR1.001[1.000-1.003],P=0.034),高白蛋白(OR1.91[1.05-3.48],P=0.034)和活检纤维化低(OR0.54[0.33-0.91],P=0.020)预测完全反应。
■AIH是最常见的AILD,其次是重叠综合征,我们队列中的PSC和PBC。在60%的AIH患者中观察到对免疫抑制的生化反应&组织病理学上的低纤维化评分预测完全反应。
UNASSIGNED: Autoimmune liver disease (AILD) comprises of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) with a spectrum of overlap amongst the three. We analyzed the spectrum and treatment outcomes of patients with AILD presenting to a tertiary care center in India.
UNASSIGNED: A retrospective analysis of AILD patients from June 2008 to April 2021 was performed. The diagnosis was based on clinical, biochemical, imaging, serological, and histological characteristics. Eligible patients received treatment depending on the disease stage. Biochemical response to treatment was defined as normalization of AST, ALT, bilirubin, and immunoglobulin G levels at 6 months in AIH, normalization of total bilirubin and/or albumin at 1 year in PBC and decrease in alkaline phosphatase (ALP) levels by 40% in PSC.
UNASSIGNED: Two hundred seventy-five patients were analyzed. AIH (58.54%) was most common, followed by an overlap of AIH-PBC (24%) and AIH-PSC (6.54%), PSC (6.18%), and PBC (4.72%). Most patients presented in 3rd or 4th decade, except PBC which occurred predominantly in 5th decade. The majority of patients were females (72.72%). Jaundice was the most common presentation seen in 60% of patients. Cirrhosis was present in 57.47% of patients. Patients with overlap had more pruritus (54.76 vs 6.83%), fatigue (63.1% vs 49.7%), hepatomegaly (52.4% vs 25.5%), and higher ALP (80.9% vs 37.7%) than patients with AIH alone. Acute presentation was seen in 33 patients (13.5%) with most having AIH flare. Five patients had acute liver failure (ALF) and 9 had acute-on-chronic liver failure (ACLF). ALF was associated with 80% mortality while 55.56% of patients with ACLF had a complete biochemical response to immunosuppression. Among patients with AIH and/or overlap who received immunosuppression, a complete biochemical response to immunosuppression was seen in 60.69% of patients. High ALT (OR 1.001 [1.000-1.003], P = 0.034), high albumin (OR 1.91 [1.05-3.48], P = 0.034) and low fibrosis on biopsy (OR 0.54 [0.33-0.91], P = 0.020) predicted complete response.
UNASSIGNED: AIH is the most common AILD followed by overlap syndromes, PSC and PBC in our cohort. Biochemical response to immunosuppression is seen in 60% of patients with AIH & low fibrosis score on histopathology predicts a complete response.