Experience in the management of children with Autoimmune Hepatitis attended at Hospital María, Especialidades Pediátricas

Abstract

Introduction: Autoimmune Hepatitis (AIH) a rare disease. Defined as a chronic, progressive inflammatory liver disease characterized by inflammation and fibrosis of the liver parenchyma and the intra-and/or extrahepatic biliary tree associated with the presence of autoantibodies and elevation of immunoglobulin G (IgG). The clinical presentation is variable. The diagnosis requires the combination of clinical, laboratory, immunological and histological criteria presented in the diagnostic guide of the International Autoimmune Hepatitis Group (IAIHG). Honduras has few publications on the frequency of HAI, so this study was carried out to describe the experience in the management of patients with HAI at the Hospital María, Pediatric Specialties (HMEP) from 2017-2022. Case description: 19 cases were found; the mean age was 10 years (standard deviation SD of 4.6). The female sex was the most affected. In 10.5% (2/19) of patients, a first degree relative with autoimmune disease was identified. 42.1% (8/19) had comorbidities of 50% (4/8) suffered from Systemic Lupus Erythematosus. The initial levels were found to be high and the biopsy performed in all cases was of typical histology for AIH in 63.5% (12/19) and 36.8% (7/19) compatible for AIH. All patients were treated with a combination of oral steroid and immunosuppressant mainly azathioprine. No mortality was recorded. Conclusion: AIH in pediatrics should be suspected and diagnostic aid criteria applied. A favorable clinical response was obtained in this group of patients.