Hospital Sírio Libanês, Hospital Menino Jesus, Grupo de Hepatologia e Transplante Pediátrico, São Paulo, SP, Brazil.
Hospital de Base do Distrito Federal, Hospital da Criança de Brasília, Departamento de Gastroenterologia e Hepatologia, Brasília, DF, Brazil.
J Pediatr (Rio J). 2019 Jul-Aug;95(4):419-427. doi: 10.1016/j.jped.2018.04.007. Epub 2018 May 30.
This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis.
The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments.
Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%).
In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.
本项大型长期随访研究旨在评估儿童和青少年自身免疫性肝炎的临床表现、实验室检查结果、组织学特征、治疗方法和转归。
回顾性分析 828 例自身免疫性肝炎患儿和青少年的病历资料。采用问卷形式收集患者的临床资料、生化和组织学检查结果及治疗方案等匿名数据。
所有患者中，89.6%为自身免疫性肝炎 1 型，10.4%为自身免疫性肝炎 2 型。两组患者均以女性为主。自身免疫性肝炎 1 型和自身免疫性肝炎 2 型患者的中位起病年龄分别为 111.5（6；210）和 53.5（8；165）个月。急性起病分别占 56.1%和 58.8%，隐匿起病分别占 43.9%和 41.2%。自身免疫性肝炎 2 型发生肝衰竭的风险是自身免疫性肝炎 1 型的 1.6 倍。自身免疫性肝炎 1 型和自身免疫性肝炎 2 型患者中分别有 3.6%和 10.6%发生暴发性肝衰竭，自身免疫性肝炎 2 型的风险是自身免疫性肝炎 1 型的 3.1 倍。自身免疫性肝炎 1 型患者的γ球蛋白和免疫球蛋白 G 水平显著升高，而自身免疫性肝炎 2 型患者的免疫球蛋白 A 和 C3 水平较低。22.4%的患者发生肝硬化，76.2%患者生化缓解。累计生存率为 93.0%。共 4.6%的患者行肝移植，6.9%患者死亡（自身免疫性肝炎 1 型：7.5%；自身免疫性肝炎 2 型：2.4%）。
在本项巴西儿童和青少年大型临床系列研究中，自身免疫性肝炎 1 型更为常见，且自身免疫性肝炎 2 型患者疾病缓解率更高，对治疗的反应更早。自身免疫性肝炎 1 型患者死亡风险更高。
