■自身免疫性肝炎表现为慢性急性肝衰竭(AIH-ACLF)是一种新颖的实体,其临床病程和管理数据有限。我们评估了AIH-ACLF患者的结果,没有肝外器官功能障碍/衰竭时给予类固醇。
■在此回顾性分析中,临床资料,实验室参数,我们计算了AIH-ACLF患者在基线时的肝活检指数和预后评分,如终末期肝病模型(MELD)和Child-Turcotte-Pugh(CTP)评分,并对不同级别的事件感染和无移植生存率进行了比较.主要结果是90天无移植存活。评估生化缓解,并确定了终点的预测因子。
■纳入了29例AIH-ACLF患者,中位随访时间为4个月。90天和180天的无移植生存率为55.2[95%置信区间(CI):39.7-76.6]%和30.2(95%CI:16.7-54.6)%,分别,是在类固醇上获得的。3例患者(10.3%)接受了肝移植,而16例(55.2%)死亡。12例患者出现感染(41.3%),导致预后评分恶化,新发器官功能障碍/衰竭和11例死亡。无移植幸存者组中的10名患者中有7名(70%)在随访中获得了生化缓解。MELD评分<24(敏感性:68.4%;特异性:80%)和CTP<11(敏感性:78.9%;特异性:90%)对生存有最好的预测价值,除了在2周时MELD评分降低(敏感性:78.9%;特异性:70%)。
■AIH-ACLF患者尽管接受了类固醇治疗,但其病程仍有病态。具有良好基线预后评分的无肝外器官衰竭的患者可以在2周内密切监测MELD变化的情况下给予类固醇。
UNASSIGNED: Autoimmune hepatitis presenting as acute on chronic liver failure (AIH-ACLF) is a novel entity with limited data on clinical course and management. We assessed outcomes in patients of AIH-ACLF with no extrahepatic organ dysfunction/failure when administered steroids.
UNASSIGNED: In this retrospective analysis, clinical data, laboratory parameters, liver biopsy indices and prognostic scores such as model for end-stage liver disease (MELD) and Child-Turcotte-Pugh (CTP) scores at baseline were computed for patients with AIH-ACLF and compared across strata of incident infections and transplant-free survival. The primary outcome was 90-day transplant-free survival. Biochemical remission was assessed, and predictors of end points were identified.
UNASSIGNED: Twenty-nine patients of AIH-ACLF were included with a median follow-up of 4 months. The 90- and 180-day transplant-free survival rates of 55.2 [95% confidence interval (CI): 39.7-76.6]% and 30.2(95% CI: 16.7-54.6)%, respectively, were attained on steroids. Three patients (10.3%) underwent liver transplant while 16 (55.2%) deaths occurred. Infections developed in 12 patients (41.3%), leading to worsening prognostic scores, new onset organ dysfunction/failure and 11 deaths. Seven of ten patients (70%) in the transplant-free survivor group attained biochemical remission on follow-up. The MELD score<24 (sensitivity: 68.4%; specificity: 80%) and CTP<11 (sensitivity: 78.9%; specificity: 90%) had best predictive value for survival, in addition to decrease in the MELD score at 2 weeks (sensitivity: 78.9%; specificity: 70%).
UNASSIGNED: Patients with AIH-ACLF have a morbid disease course despite treatment with steroids. Patients with no extrahepatic organ failure with good baseline prognostic scores may be administered steroids with close monitoring for change in MELD over 2 weeks.