Pediatr. praxi. 2023;24(3):158-162 | DOI: 10.36290/ped.2023.048

Autoimmune liver disease in childhood

MUDr. Lucie Gonsorčíková, Ph.D.1, MUDr. David Bauer1, doc. MUDr. Eva Sticová, Ph.D.2, MUDr. Radim Vyhnánek1
1 Pediatrická klinika 1. LF UK a Fakultní Thomayerovy nemocnice, Praha
2 Pracoviště klinické a transplantační patologie IKEM, Praha

Autoimmune liver disease in children can be classified as: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC) and de novo AIH after liver transplantation (plasma cell rich rejection). When left untreated, these disorders are progressive in nature and can result in cirrhosis and terminal liver failure. They are characterised by presence of circulating autoantibodies, high levels of IgG class imunoglobulins and inflammatory changes on liver histology. There are two main subtypes of AIH depending on the type of specific autoantibodies detected: type 1 and type 2. AIH has a very wide spectrum of symptoms ranging from being asymptomatic to an acute severe fulminant disease. These liver disorders respond well to theimmunospuresive therapy which must be started as early as possible.

Keywords: autoimmune hepatitis, sclerosing cholangitis, immunosupresive therapy.

Accepted: June 8, 2023; Published: June 15, 2023  Show citation

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Gonsorčíková L, Bauer D, Sticová E, Vyhnánek R. Autoimmune liver disease in childhood. Pediatr. praxi. 2023;24(3):158-162. doi: 10.36290/ped.2023.048.
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