Angelo Di Giorgio1, Nedim Hadzic2, Anil Dhawan2, Maesha Deheragoda3, Michael A Heneghan4, Diego Vergani5, Giorgina Mieli-Vergani6, Marianne Samyn2. 1. Pediatric Liver, Gastrointestinal, and Nutrition Center, King's College Hospital, London, United Kingdom; Pediatric Liver, Gastrointestinal, and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Italy. Electronic address: adigiorgio@asst-pg23.it. 2. Pediatric Liver, Gastrointestinal, and Nutrition Center, King's College Hospital, London, United Kingdom. 3. Histopathology Department, Institute of Liver Studies King's College Hospital London, United Kingdom. 4. Institute of Liver Studies, King's College Hospital, London, United Kingdom. 5. King's College London Faculty of Life Sciences and Medicine, Institute of Liver Studies, Mowat Labs King's College Hospital, London, United Kingdom. 6. Pediatric Liver, Gastrointestinal, and Nutrition Center, King's College Hospital, London, United Kingdom; King's College London Faculty of Life Sciences and Medicine, Institute of Liver Studies, Mowat Labs King's College Hospital, London, United Kingdom.
Abstract
OBJECTIVES: To report baseline features and long-term medical/social outcomes of juvenile autoimmune liver disease, including autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC), managed in a single tertiary center. STUDY DESIGN: Retrospective study of children diagnosed in 2000-2004 with AIH/ASC followed up to date. Patients with abnormal cholangiogram were classified as ASC. Presentation and outcome features were compared. RESULTS: Eighty-three children were included (42 female, median age 12.1 years [8.5-14.1 years], AIH = 54, ASC = 29). Most (65%) had antinuclear and/or anti-smooth muscle autoantibodies; 6% presented with acute liver failure; 29% had histologic evidence of cirrhosis. The 1999 and simplified International Autoimmune Hepatitis Group criteria failed to diagnose up to 26% of patients with AIH and 48% with ASC, and the proposed the European Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria were accurate. Response to treatment was excellent with 95% achieving normal transaminase levels. During follow-up, 31% had at least 1 relapse episode; 3 patients with AIH developed cholangiopathy and 5 patients with ASC developed progressive bile duct injury. At last follow-up (median of 14.5 years, 10.4-16.8), 99% were alive, 11 underwent transplantation and 1 is listed for transplant. Five-, 10-, and 15-year transplant-free survival rates were 95%, 88%, and 83%; patients with ASC and those relapsing being more likely to require transplant. Social outcome was excellent with 93% in employment/education. CONCLUSIONS: Seamless management of juvenile autoimmune liver disease leads to excellent clinical and social outcomes. Despite good response to immunosuppressive treatment, patients with ASC have a worse prognosis than those with AIH. Diagnostic models developed for adults are unsatisfactory to correctly diagnose juvenile autoimmune liver disease. Published by Elsevier Inc.
OBJECTIVES: To report baseline features and long-term medical/social outcomes of juvenile autoimmune liver disease, including autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (ASC), managed in a single tertiary center. STUDY DESIGN: Retrospective study of children diagnosed in 2000-2004 with AIH/ASC followed up to date. Patients with abnormal cholangiogram were classified as ASC. Presentation and outcome features were compared. RESULTS: Eighty-three children were included (42 female, median age 12.1 years [8.5-14.1 years], AIH = 54, ASC = 29). Most (65%) had antinuclear and/or anti-smooth muscle autoantibodies; 6% presented with acute liver failure; 29% had histologic evidence of cirrhosis. The 1999 and simplified International Autoimmune Hepatitis Group criteria failed to diagnose up to 26% of patients with AIH and 48% with ASC, and the proposed the European Society for Pediatric Gastroenterology, Hepatology and Nutrition criteria were accurate. Response to treatment was excellent with 95% achieving normal transaminase levels. During follow-up, 31% had at least 1 relapse episode; 3 patients with AIH developed cholangiopathy and 5 patients with ASC developed progressive bile duct injury. At last follow-up (median of 14.5 years, 10.4-16.8), 99% were alive, 11 underwent transplantation and 1 is listed for transplant. Five-, 10-, and 15-year transplant-free survival rates were 95%, 88%, and 83%; patients with ASC and those relapsing being more likely to require transplant. Social outcome was excellent with 93% in employment/education. CONCLUSIONS: Seamless management of juvenile autoimmune liver disease leads to excellent clinical and social outcomes. Despite good response to immunosuppressive treatment, patients with ASC have a worse prognosis than those with AIH. Diagnostic models developed for adults are unsatisfactory to correctly diagnose juvenile autoimmune liver disease. Published by Elsevier Inc.
Authors: Angelo Di Giorgio; Anna Tulone; Emanuele Nicastro; Lorenzo Norsa; Aurelio Sonzogni; Lorenzo D'Antiga Journal: World J Hepatol Date: 2021-12-27
Authors: Emilia Shin; Kathleen B Schwarz; Lorraine V Jones-Brando; Liliana D Florea; Sarven Sabunciyan; Laura Delong Wood; Robert H Yolken Journal: J Pediatr Gastroenterol Nutr Date: 2022-06-27 Impact factor: 3.288