自身免疫性肝炎所致肝衰竭的临床表现和组织病理学特征

摘要/Abstract

摘要： 目的分析不同临床结局自身免疫性肝炎相关肝功能衰竭(AIH-LF)患者临床表现和组织病理学特征。方法回顾收集2010年1月—2022年6月期间在盐城市第一人民医院接受治疗的AIH-LF患者23例,其中男性3例、女性20例,年龄44(30,53)岁。依据病情分为AIH-LF缓解与进展组,比较两组临床资料与组织病理学特征。结果临床资料方面,AIH-LF缓解组AIH病程>6个月、确诊肝衰竭间隔时间为4例(33.3%)、11(2, 17)个月,均显著低于AIH-LF进展组[7例(63.6%)、23(4, 51)个月,P<0.05];AIH-LF缓解组亚急性、慢加急性肝衰竭分别为8例(66.7%)、3例(25.0%),与AIH-LF进展组[2例(18.2%)、8例(72.7%)]比较,差异具有统计学意义(P<0.05);AIH-LF缓解与进展组肝硬化例数为1例(8.3%)、5例(45.4%),差异具有统计学意义(P<0.05);AIH-LF缓解组应用类固醇激素治疗9例(75.0%),显著高于AIH-LF进展组[3例(27.3%),P<0.05];AIH-LF缓解组厌食、感染、肾功能不全及消化道出血分别为3例(25.0%)、4例(33.3%)、0(0)及0(0),均显著低于AIH-LF进展组[8例(72.7%)、9例(81.8%)、4例(36.4%)及4例(36.4%),P<0.05];AIH-LF缓解与进展组AFP水平为104(42, 176)ng/mL、55(4, 122)ng/mL,差异具有统计学意义(P<0.05);AIH-LF缓解组IgG阳性例数为1例(8.3%),显著低于AIH-LF进展组[5例(45.4%),P<0.05]。组织病理学特征方面,AIH-LF缓解组界面性肝炎为10例(83.3%),显著高于AIH-LF进展组[4例(36.4%),P<0.05];AIH-LF缓解组胆管损伤及F3-F4期肝纤维化例数为1例(8.3%)及2例(16.7%),分别显著低于AIH-LF进展组[6例(54.5%)及7例(63.6%),P<0.05]。结论虽然AIH-LF发病率并不高,但可造成较高的死亡率。不同的临床特征预示着临床结局的差异,这对临床工作有着重要意义。AIH-LF进展患者AIH病程长、肝硬化多见,常伴有厌食、感染、肾功能不全、消化道出血等症状,同时组织病理学多以胆管损伤、显著肝纤维化为特点。

关键词: 自身免疫性肝炎, 肝功能衰竭, 类固醇激素, 肝纤维化

Abstract: Objective To analyze the clinical manifestations and histopathological features of autoimmune hepatitis-related liver failure (AIH-LF) patients with different clinical outcomes.Methods Twenty-three patients with AIH-LF admited in the first people’s hospital of Yancheng city from January 2010 to June 2022 were collected retrospectively, including 3 males and 20 females, aged 44 (30, 53) years. According to the condition, they were divided into an AIH-LF remission group and a progression group. The clinical data and histopathological features of the two groups of patients were compared. Results In terms of clinical data, the duration of AIH patients in the remission group was more than 6 months, and the number of cases and the time interval of the diagnosis of liver failure was 4 cases (33.3%) and 11 (2, 17) months, which were significantly lower than those in the progressive group [7 cases (63.6%) and 23 (4, 51) months, P<0.05]. There were 8 cases (66.7%) and 2 cases (18.2%) of subacute, acute on chronic liver failure in AIH-LF remission group, compared with 2 cases (18.2%) and 8 cases (72.7%) in AIH-LF progression group, the difference was statistically significant (P<0.05). There were 1 case (8.3%) and 5 cases (45.4%) of cirrhosis in AIH-LF remission and progression group, and the difference was statistically significant (P<0.05). 9 patients (75.0%) in AIH-LF remission group were treated with steroid hormone, which was significantly higher than that in AIH-LF progression group [3 patients (27.3%), P<0.05]. Anorexia, infection, renal insufficiency and gastrointestinal bleeding in AIH-LF remission group were 3 cases (25.0%), 4 cases (33.3%), 0 (0) and 0 (0), respectively, which were significantly lower than those in AIH-LF progression group [8 cases (72.7%), 9 cases (81.8%), 4 cases (36.4%) and 4 cases (36.4%), P<0.05]. The levels of AFP in the remission and progression group of AIH-LF were 104 (42, 176) ng/mL and 55 (4, 122) ng/mL, and the difference was statistically significant (P<0.05). The number of IgG positive cases in AIH-LF remission group was 1 case (8.3%), which was significantly lower than that in AIH-LF progression group [5 cases (45.4%), P<0.05]. As for the histopathological features, there were 10 cases (83.3%) of interfacial hepatitis in AIH-LF remission group, which were significantly higher than those of 4 cases (36.4%) in AIH-LF progression group [P<0.05]. The cases of bile duct injury and F3-F4 hepatic fibrosis in AIH-LF remission group were 1 case (8.3%) and 2 cases (16.7%), respectively, which were significantly lower than those of 6 cases (54.5%) and 6 cases (54.5%) in the AIH-LF progression group [P<0.05]. Conclusion Although the incidence of AIH-LF is not high, it causes high mortality. Different clinical features indicate different clinical outcomes, which is of great significance for clinical work. Patients with advanced AIH-LF have a long course of AIH and liver cirrhosis, often accompanied by anorexia, infection, renal insufficiency, gastrointestinal bleeding and other symptoms. At the same time, histopathology is characterized by bile duct injury and significant liver fibrosis.

Key words: Autoimmune hepatitis, Liver failure, Steroid, Hepatic fibrosis

王姝姝, 王树人. 自身免疫性肝炎所致肝衰竭的临床表现和组织病理学特征[J]. 肝脏, 2023, 28(9): 1037-1040.

WANG Shu-shu, WANG Shu-ren. Clinical manifestations and histopathological features of liver failure caused by autoimmune hepatitis[J]. Chinese Hepatolgy, 2023, 28(9): 1037-1040.

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