Open AccessEvolving Practice and Changing Phenotype in Pediatric Autoimmune Liver Disease
Author(s) -
Singh Harveen,
Balouch Fariha,
Noble Charlton,
Lewindon Peter
Publication year - 2018
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000001927
Subject(s) - medicine , phenotype , autoimmune disease , autoimmune hepatitis , liver disease , disease , immunology , pathology , genetics , gene , biology
Objectives: 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. Methods: Retrospective chart review was conducted from January 2000 to 2016. Data were divided into two 8‐year cohorts, CI and C2. Results: 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 ulcerative colitis (UC). Colectomy was required in 3 children with large duct PSC‐IBD. Conclusions: PSC and ASC are increasing in relevance along with IBD and reflect increasing performance of magnetic resonance cholangio‐pancreatography and colonoscopy. Large duct PSC and ASC with IBD are risk factors for colectomy and along with female gender, for liver transplant.