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Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study.

Abstract：

BACKGROUND:The incidence of very early onset inflammatory bowel disease (VEOIBD) is increasing, yet the phenotype and natural history of VEOIBD are not well described. METHODS:We performed a retrospective cohort study of patients diagnosed with VEOIBD (6 years of age and younger) between 2008 and 2013 at 25 North American centers. Eligible patients at each center were randomly selected for chart review. We abstracted data at diagnosis and at 1, 3, and 5 years after diagnosis. We compared the clinical features and outcomes with VEOIBD diagnosed younger than 3 years of age with children diagnosed with VEOIBD at age 3 to 6 years. RESULTS:The study population included 269 children (105 [39%] Crohn's disease, 106 [39%] ulcerative colitis, and 58 [22%] IBD unclassified). The median age of diagnosis was 4.2 years (interquartile range 2.9-5.2). Most (94%) Crohn's disease patients had inflammatory disease behavior (B1). Isolated colitis (L2) was the most common disease location (70% of children diagnosed younger than 3 years vs 43% of children diagnosed 3 years and older; P = 0.10). By the end of follow-up, stricturing/penetrating occurred in 7 (6.6%) children. The risk of any bowel surgery in Crohn's disease was 3% by 1 year, 12% by 3 years, and 15% by 5 years and did not differ by age at diagnosis. Most ulcerative colitis patients had pancolitis (57% of children diagnosed younger than 3 years vs 45% of children diagnosed 3 years and older; P = 0.18). The risk of colectomy in ulcerative colitis/IBD unclassified was 0% by 1 year, 3% by 3 years, and 14% by 5 years and did not differ by age of diagnosis. CONCLUSIONS:Very early onset inflammatory bowel disease has a distinct phenotype with predominantly colonic involvement and infrequent stricturing/penetrating disease. The cumulative risk of bowel surgery in children with VEOIBD was approximately 14%-15% by 5 years. These data can be used to provide anticipatory guidance in this emerging patient population.

journal_name

Inflamm Bowel Dis

journal_title

Inflammatory bowel diseases

authors

Kerur B,Benchimol EI,Fiedler K,Stahl M,Hyams J,Stephens M,Lu Y,Pfefferkorn M,Alkhouri R,Strople J,Kelsen J,Siebold L,Goyal A,Rosh JR,LeLeiko N,Van Limbergen J,Guerrerio AL,Maltz R,Karam L,Crowley E,Griffiths A,H

doi

10.1093/ibd/izaa080

subject

Has Abstract

pub_date

2020-05-09 00:00:00

eissn

1078-0998

issn

1536-4844

pii

5835042

pub_type

杂志文章

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Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study.