Autoimmune hepatitis

Aim: To establish diagnostic criteria, incidence, treatment modalities, genetics and long-term outcome of juvenile autoimmune liver disease (AILD)

Description: juvenile AILD includes autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC). As ASC has the same immunological profile of AIH type 1 (positivity for anti-nuclear and/or anti smooth muscle antibodies), a cholangiogram at presentation is necessary to distinguish between the two conditions

History: The ESPGHAN AILD Group is part of the International Autoimmune Hepatitis Group (IAIHG). The IAIHG was established in 1992 and meets twice yearly. Its original task was of issuing diagnostic criteria for adult AIH (Johnson PJ, et al.  Meeting report: International Autoimmune Hepatitis Group. Hepatology. 993;18:998-1005; Alvarez F, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999;31:929-38; Hennes EM, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008;48:169-76). A limitation of the IAIHG scoring systems, however, is that they have been produced for adult patients and need to be adapted to children. Though useful for the diagnosis of autoimmune liver disease, they do not allow distinction between AIH and ASC.

Activity: The group meets at the ESPGHAN annual conference, as well as the EASL and AASLD annual conferences within the IAIHG meetings

Projects: To establish diagnostic criteria, incidence, treatment modalities, genetics and long-term outcome of juvenile AILD within the IAIHG

Papers/Guidelines: Mieli-Vergani G, et al. J Pediatr Gastroenterol Nutr 2009;49:158-164. Autoimmune Hepatitis Paper

Networking: Within the IAIHG the group is networking with 45 centres: 22 in Europe (Italy: 9, UK: 3, Germany: 3, France: 2, Poland: 1, Denmark:1, Sweden: 1, Spain;1); 10 in Turkey; 7 in Israel; 4 in the USA; 1 in Brazil; 1 in Egypt

Meetings: EASL Monothematic Conference: Autoimmune Hepatitis. Venue and dates: London, UK, September 3-5, 2015

www.easl.eu/_events/easl-monothematic-conference/easl-monothematic-conference-autoimmune-hepatitis

Reports: Minutes of IAIHG meetings are available on request