https://www.ncbi.nlm.nih.gov/pubmed/29856944

J Pediatr (Rio J). 2018 May 29. pii: S0021-7557(17)31099-9. doi: 10.1016/j.jped.2018.04.007. [Epub ahead of print]
Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes.

Porta G1, Carvalho E2, Santos JL3, Gama J4, Borges CV5, Seixas RBPM6, Ferreira AR7, Miura IK5, Silveira TR8, Silva LR9, Fagundes EDT7, Bellomo-Brandao MA10, Sawamura R11, Vieira SM12, Melere MU8, Marques CDF9, Pugliese RP5, Danesi VL5, Porta A5, Marsillac ME13, Valladares MA14, Menezes DG15, Kieling C12, Paula MN16, Vasconcelos JR17, Ferreira CT8, Perin N18, Resende LR19, Maia J20, Tommaso AMA10, Hessel G10.

Abstract

OBJECTIVE:
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.

METHODS:
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.

RESULTS:
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%).

CONCLUSIONS:
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.