儿童自身免疫性急性肝衰竭和血清阴性自身免疫性肝病：它们与经典疾病有何不同？

Autoimmune acute liver failure and seronegative autoimmune liver disease in children: Are they different from classical disease?

作者信息

Jain Vikas, Srivastava Anshu, Yachha Surender K, Kumari Niraj, Kathuria Rachana, Sarma Moinak S, Poddar Ujjal, Krishnani Narendra

机构信息

Departments of aPediatric Gastroenterology bPathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

出版信息

Eur J Gastroenterol Hepatol. 2017 Dec;29(12):1408-1415. doi: 10.1097/MEG.0000000000000975.

DOI:10.1097/MEG.0000000000000975

PMID:28914695

Abstract

OBJECTIVES

Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD).

MATERIALS AND METHODS

Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available.

RESULTS

Seventy children [AI-ALF=15 and AI-non-ALF=55 (SN-AILD=11, SP-AILD=44)] were evaluated. Age at presentation [7 (1.2-16) vs. 9 (2-17) years] percentage of female patients (67 vs. 62%), and AILD type (type II, 53 vs. 31%) were similar in AI-ALF and AI-non-ALF patients], respectively. 8/15 AI-ALF cases were treated with steroids (improved-4, liver transplant-1, and death-3) and 7/15 died before initiation of therapy. Hepatic encephalopathy (100 vs. 16.3%; P<0.001), massive hepatic necrosis (60 vs. 0%; P<0.001), and higher pediatric end-stage liver disease [n=53, 29.9 (13.1-56.9) vs. 9.8 (-10-28.7) P<0.001], model for end-stage liver disease [n=17, 38.5 (24-46) vs. 18 (6-24); P=0.005], and Child-Turcotte-Pugh [n=70, 13 (8-13) vs. 9 (5-13); P<0.001] scores were features of AI-ALF. Poorer response to immunosuppression (4/8 vs. 48/55; P=0.02) and higher mortality (11/15 vs. 4/55; P=0.0001) were seen in AI-ALF than in AI-non-ALF patients. Clinicolaboratory profile, therapeutic response, and outcome were similar in SN-AILD and SP-AILD.

CONCLUSION

AI-ALF is characterized by poorer liver function, lower response to immunosuppression, and higher mortality compared with SP or SN AI-non-ALF, which are similar.

摘要

目的

自身免疫性急性肝衰竭（AI-ALF）和血清阴性自身免疫性肝病（SN-AILD）是自身免疫性肝病（AILD）的两种罕见类型。我们比较了AI-ALF与自身免疫性非急性肝衰竭（AI-non-ALF）的临床特征及预后，以及SN-AILD与血清阳性自身免疫性肝病（SP-AILD）的临床特征及预后。

材料与方法

纳入并比较诊断为AI-ALF和AI-non-ALF的儿童患者，包括SN-AILD和SP-AILD患者。AI-non-ALF根据简化诊断标准确诊，AI-ALF根据儿童急性肝衰竭研究组标准确诊，伴有自身抗体阳性、排除其他病因、免疫球蛋白G升高以及必要时的组织学检查结果。

结果

共评估了70例儿童患者[AI-ALF = 15例，AI-non-ALF = 55例（SN-AILD = 11例，SP-AILD = 44例）]。AI-ALF和AI-non-ALF患者的就诊年龄[7（1.2 - 16）岁 vs. 9（2 - 17）岁]、女性患者比例（67% vs. 62%）以及AILD类型（II型，53% vs. 31%）相似。15例AI-ALF患者中有8例接受了类固醇治疗（病情改善4例、肝移植1例、死亡3例），15例中有7例在开始治疗前死亡。肝性脑病（100% vs. 16.3%；P < 0.001）、大片肝坏死（60% vs. 0%；P < 0.001）以及更高的儿童终末期肝病评分[n = 53，29.9（13.1 - 56.9） vs. 9.8（-10 - 28.7）；P < 0.001]、终末期肝病模型评分[n = 17，38.5（24 - 46） vs. 18（6 - 24）；P = 0.005]和Child-Turcotte-Pugh评分[n = 70，13（8 - 13） vs. 9（5 - 13）；P < 0.001]是AI-ALF的特征。与AI-non-ALF患者相比，AI-ALF患者对免疫抑制治疗的反应较差（4/8 vs. 48/55；P = 0.02）且死亡率更高（11/15 vs. 4/55；P = 0.0001）。SN-AILD和SP-AILD的临床实验室特征、治疗反应及预后相似。