Autoimmune hepatitis (AIH), a progressive chronic liver disease, is typified by periportal inflammation, bridging necrosis, liver cell rosetting, and marked plasma cell infiltration. To study the disease in pediatric population, Porta and colleagues conducted a trial to assess clinical presentation, laboratory findings, histological profile, treatments, and outcomes of 828 children and adolescents with AIH.

Results demonstrated that among the patients, 89.6% were suffering from AIH-1 and 10.4% had AIH-2. Moreover, female patients were more prevalent than male patients in both the groups. The median age at symptom onset was 111.5 months in AIH-1 patients and 53.5 months in patients with AIH-2. Acute clinical onset was observed in 56.1% and 58.8% whereas insidious symptoms were reported in 43.9% and 41.2% of the patients with AIH-1 and AIH-2, respectively. Out of total patients, 3.6% of patients with AIH-1 had fulminant hepatic failure while 10.6% of the patients with AIH-2 had it, suggestive of the evidence that the risk of hepatic failure was 3.1-fold higher for AIH-2. Besides, patients with AIH-1 had higher levels of gamma globulin and immunoglobulin G levels, whereas immunoglobulin A and C3 levels were lower in AIH-2. The progression to cirrhosis was found in 22.4% of the patients. A total of 4.6% underwent liver transplantation, and 6.9% died with actual survival rate of 93.0%.

Therefore, it was concluded that AIH-1 was more common in children and adolescents with higher risk of death. Moreover, higher disease remission rates with earlier response to treatment were found in patients with AIH-2.

Source:  Porta G, Carvalho E, Santos JL, et al. Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes. J Pediatr (Rio J). 2019;95(4):419-427.