Clinical spectrum and outcomes of autoimmune hepatitis in children: a quaternary centre experience

Abstract

Objectives: To analyse paediatric autoimmune hepatitis with respect to disease severity, treatment response, and clinical outcomes, including progression to liver failure and transplant survival.

Method: The retro-prospective study was conducted at the Pakistan Kidney and Liver Institute and Research Centre, Lahore, Pakistan, and comprised data from July 2019 to December 2024 of paediatric patients aged <15 years diagnosed with autoimmune hepatitis. Demographic characteristics, clinical manifestations as well as laboratory and imaging findings were noted. Data was analysed using SPSS 27.

Results: Of the 56 patients, 30(53.6%) were girls with mean age 9.94±2.63 years, and 26(46.4%) were boys with mean age 9.24±2.90 years. Autoimmune hepatitis type 1 was the predominant type 46(82.1%), while type 2 was found in 1(1.8%) patient and 9(16.1%) were seronegative. Jaundice was the most common symptom found in 54(96.4%) followed by ascites 28(50%) and abdominal pain 18(32.1%).  Histopathology of liver biopsies revealed features consistent with autoimmune hepatitis including statistically significant hepatocyte rosette formation (p-value= 0.100). Overall, 8 (14.3%) patients required liver transplantation.

Conclusion: The clinical spectrum of autoimmune hepatitis and its outcomes in children indicated there were significant diagnostic and therapeutic challenges that needed to be overcome.

Key Words: Autoimmune diseases, Autoantibodies, Immunosuppressive agents, Liver biopsy, Liver transplantation.