Can 1-year-old children develop autoimmune hepatitis?

From the Guidelines

Yes, one-year-old children can develop autoimmune hepatitis, though it is rare in this age group. Autoimmune hepatitis is a chronic liver disease where the body's immune system attacks liver cells, causing inflammation and damage. In young children, diagnosis involves blood tests showing elevated liver enzymes, presence of specific autoantibodies (ANA, SMA, anti-LKM), and a liver biopsy.

Key Points to Consider

• The disease can affect individuals of any age, including small infants and octogenarians, as stated in the EASL clinical practice guidelines 1.
• Treatment typically includes immunosuppressive medications such as prednisone (starting at 1-2 mg/kg/day) and often azathioprine (1-2 mg/kg/day) as a steroid-sparing agent, as mentioned in the diagnosis and management of autoimmune hepatitis study 1.
• Early diagnosis and treatment are crucial to prevent progression to cirrhosis, and the disease requires long-term management with regular monitoring of liver function and medication side effects.
• The exact cause remains unclear but likely involves genetic predisposition and environmental triggers, as noted in the EASL clinical practice guidelines 1.
• Parents should watch for symptoms like jaundice (yellowing of skin/eyes), fatigue, abdominal pain, or enlarged liver, though some children may be asymptomatic with only abnormal liver tests.

Important Considerations for Treatment and Management

• The response to treatment with corticosteroids with or without azathioprine is generally excellent in children, with normalization of liver tests noted after 6-9 months of therapy in 75%-90% of cases, as reported in the diagnosis and management of autoimmune hepatitis study 1.
• Liver transplantation is the only effective treatment for patients with severe autoimmune hepatitis who fail to respond to immunosuppressive therapy or who develop advanced decompensated disease despite treatment, as stated in the AASLD practice guidelines 1.
• The outcome after liver transplantation is excellent, with reported 5- and 10-year survival rates of more than 75% in adults, and the risk of recurrent disease is estimated to be 10%-35%, as noted in the evaluation of the pediatric patient for liver transplantation study 1.

From the Research

Autoimmune Hepatitis in 1-Year-Olds

• Autoimmune hepatitis (AIH) is a rare progressive liver disease that can affect individuals of all ages, including children 2, 3, 4, 5, 6.
• The clinical manifestations of AIH in children may differ from those in adults, and it can present as an acute disease in children 4.
• AIH can be classified into two types: type 1, with anti-smooth muscle antibodies, and type 2, with anti-liver/kidney microsomal antibodies 4, 5.
• Children with AIH may have identifiable monogenic disorders of immune regulation, such as autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) 4.
• The diagnosis of AIH is based on a scoring system codified by an international consensus, and treatment with corticosteroids and azathioprine can induce remission in over 80% of patients 5.
• While there is no specific information on 1-year-olds with AIH in the provided studies, it is clear that AIH can affect children of all ages, including young children 6.

Key Findings

• AIH is a rare but serious cause of chronic hepatic disease in children that can lead to cirrhosis and hepatic failure 3.
• The pathogenic mechanisms of AIH remain obscure, but immunologic and genetic factors are thought to play a role 3.
• Early diagnosis and treatment with immunosuppressive therapy can improve prognosis and decrease the number of indicated liver transplantations in children with AIH 2.
• Liver transplantation may be necessary in some cases, but AIH can recur or develop de novo after liver transplantation 5, 6.