Abstract
Autoimmune liver disease (AILD) incorporates primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and autoimmune sclerosing cholangitis (ASC). ASC is a condition that includes overlap of AIH and PSC. We investigate changes in practice in relation to diagnosis and phenotype over 2 time periods. Retrospective chart review was conducted from January 2000 to 2016. Data were divided into two 8-year cohorts, CI and C2. Data were collected in 75 children, 29 in 2000-2007 (C1) and 46 in 2008-2016 (C2). Presenting AILD type was AIH in 59%, ASC in 10%, and PSC in 31%. Final AILD type was AIH in 53%, ASC in 16%, and PSC in 31%. When comparing C1 to C2, those with AIH decreased (65% vs 45%) and those with ASC increased (14% vs 18%). Use of magnetic resonance cholangio-pancreatography increased from 34% in C1 to 65% in C2. Advanced liver disease on biopsy was noted in 53% of all children at presentation. Only 5 female children progressed to liver transplant (3 ASC-IBD [inflammatory bowel disease]; 1 PSC-IBD; 1 AIH). Colonoscopy performance increased from 48% in C1 to 63% in C2 with diagnosis of AILD-IBD increasing from 31% to 52%. Right-sided disease was present in 46% and macroscopic rectal sparing in 36% of those with ulcerat...Continue Reading
References
Mar 7, 2001·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·G V GregorioG Mieli-Vergani
Oct 11, 2001·Journal of Pediatric Gastroenterology and Nutrition·W A FaubionJ Perrault
Jun 18, 2003·The American Journal of Gastroenterology·Kelly W BurakKeith D Lindor
Jun 28, 2003·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Ariel E FeldsteinPaul Angulo
Feb 5, 2008·Clinics in Liver Disease·Diego Vergani, Giorgina Mieli-Vergani
Jan 6, 2009·Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association·Tamir MilohNanda Kerkar
Jul 4, 2009·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Maïté LewinChristophe Corpechot
Jan 27, 2010·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Roger ChapmanUNKNOWN American Association for the Study of Liver Diseases
Feb 10, 2010·Gastroenterology·Francis A FarrayeUNKNOWN AGA Institute Medical Position Panel on Diagnosis and Management of Colorectal Neoplasia in Inflammatory Bowel Disease
Apr 16, 2010·Current Opinion in Gastroenterology·Giorgina Mieli-Vergani, Diego Vergani
Mar 30, 2011·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Steven R MartinUNKNOWN SPLIT Research Group
Apr 21, 2011·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Tamir MilohUNKNOWN Studies of Pediatric Liver Transplantation Research Group
Nov 10, 2011·The American Journal of Gastroenterology·Khalid AlswatGideon M Hirschfield
Jan 13, 2012·Inflammatory Bowel Diseases·Felipe OrdonezFrank M Ruemmele
May 21, 2013·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Mark DeneauStephen L Guthery
Mar 19, 2014·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Veena L VenkatRakesh Sindhi
Apr 24, 2014·Fetal and Pediatric Pathology·Claudia Patricia RojasMaria Matilde Rodriguez
May 7, 2014·World Journal of Hepatology·Christos TsaitasEmmanouil Sinakos
Oct 21, 2015·Pediatrics·Carolina Jiménez-RiveraFernando Alvarez
Jan 13, 2016·Journal of Pediatric Gastroenterology and Nutrition·Matteo BramuzzoUNKNOWN SIGENP IBD Group
Jan 16, 2016·Pediatric Gastroenterology, Hepatology & Nutrition·Jisun YoonKyung Mo Kim
Aug 19, 2016·United European Gastroenterology Journal·Andrea TencaKaija-Leena Kolho
Dec 17, 2016·Journal of Pediatric Gastroenterology and Nutrition·Lawrence J SaubermannBinita M Kamath
Mar 10, 2017·Gastroenterology·Tobias J WeismüllerUNKNOWN International PSC Study Group