Diagnosis and Management of Paediatric Autoimmune Liver Disease
(2018) Journal of Pediatric Gastroenterology and Nutrition — Vol. 66, n° 2, p. 345-360 (2018)
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- Paediatric autoimmune liver disease is characterised by inflammatory liver histology, circulating autoantibodies and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo AIH after liver transplantation. Two types of paediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (anti-LKM-1/anti-LC-1; AIH-2).Pertinent issues addressing the diagnosis, treatment and long term follow up were formulated by a core group of ESPGHAN members. They have commissioned the first authors with execution of this project. Initially, they have performed a systematic literature search on MEDLINE, ResearchGate and Mendeley databases over the last 30 years and produced a document focusing on prospective and retrospective studies in children. The ESPGHAN core group and ESPGHAN Hepatology Committee members voted on each recommendation, using a formal voting technique.
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Mieli-Vergani, G., Vergani, D., Baumann, U., Czubkowski, P., Debray, D., Dezsofi, A., Fischler, B., Gupte, G., Hierro, L., Indolfi, G., Jahnel, J., Smets, F., Verkade, H. J., & Hadzic, N. (2018). Diagnosis and Management of Paediatric Autoimmune Liver Disease. Journal of Pediatric Gastroenterology and Nutrition, 66(2), 345-360. https://doi.org/10.1097/MPG.0000000000001801 (Original work published 2018)