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

Basavaraj Kerur; Eric I. Benchimol; Karoline Fiedler; Marisa Stahl; Jeffrey Hyams; Michael Stephens; Ying Lu; Marian Pfefferkorn; Raza Alkhouri; Jennifer Strople; Judith Kelsen; Leah Siebold; Alka Goyal; Joel R. Rosh; Neal Leleiko; Johan Van Limbergen; Anthony L. Guerrerio; Ross Maltz; Lina Karam; Eileen Crowley; Anne Griffiths; Melvin B. Heyman; Mark Deneau; Keith Benkov; Joshua Noe; Dedrick Mouton; Helen Pappa; Joseph A. Galanko; Scott Snapper; Aleixo M. Muise; Michael D. Kappelman

doi:10.1093/ibd/izaa080

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

Basavaraj Kerur, Eric I. Benchimol, Karoline Fiedler, Marisa Stahl, Jeffrey Hyams, Michael Stephens, Ying Lu, Marian Pfefferkorn, Raza Alkhouri, Jennifer Strople, Judith Kelsen, Leah Siebold, Alka Goyal, Joel R. Rosh, Neal Leleiko, Johan Van Limbergen, Anthony L. Guerrerio, Ross Maltz, Lina Karam, Eileen CrowleyAnne Griffiths, Melvin B. Heyman, Mark Deneau, Keith Benkov, Joshua Noe, Dedrick Mouton, Helen Pappa, Joseph A. Galanko, Scott Snapper, Aleixo M. Muise, Michael D. Kappelman

School of Medicine

Research output: Contribution to journal › Article › peer-review

2 Scopus citations

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.

Original language	English (US)
Pages (from-to)	295-302
Number of pages	8
Journal	Inflammatory bowel diseases
Volume	27
Issue number	3
DOIs	https://doi.org/10.1093/ibd/izaa080
State	Published - Mar 1 2021

Keywords

VEOIBD
epidemiology
surgery

ASJC Scopus subject areas

Immunology and Allergy
Gastroenterology

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10.1093/ibd/izaa080

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Kerur, B., Benchimol, E. I., Fiedler, K., Stahl, M., Hyams, J., Stephens, M., Lu, Y., Pfefferkorn, M., Alkhouri, R., Strople, J., Kelsen, J., Siebold, L., Goyal, A., Rosh, J. R., Leleiko, N., Van Limbergen, J., Guerrerio, A. L., Maltz, R., Karam, L., ... Kappelman, M. D. (2021). Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study. Inflammatory bowel diseases, 27(3), 295-302. https://doi.org/10.1093/ibd/izaa080

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, Heyman, MB, Deneau, M, Benkov, K, Noe, J, Mouton, D, Pappa, H, Galanko, JA, Snapper, S, Muise, AM & Kappelman, MD 2021, 'Natural History of Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study', Inflammatory bowel diseases, vol. 27, no. 3, pp. 295-302. https://doi.org/10.1093/ibd/izaa080

@article{e39eb7bcb9d84298812d72dee70a0e8e,

title = "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.",

keywords = "VEOIBD, epidemiology, surgery",

author = "Basavaraj Kerur and Benchimol, {Eric I.} and Karoline Fiedler and Marisa Stahl and Jeffrey Hyams and Michael Stephens and Ying Lu and Marian Pfefferkorn and Raza Alkhouri and Jennifer Strople and Judith Kelsen and Leah Siebold and Alka Goyal and Rosh, {Joel R.} and Neal Leleiko and {Van Limbergen}, Johan and Guerrerio, {Anthony L.} and Ross Maltz and Lina Karam and Eileen Crowley and Anne Griffiths and Heyman, {Melvin B.} and Mark Deneau and Keith Benkov and Joshua Noe and Dedrick Mouton and Helen Pappa and Galanko, {Joseph A.} and Scott Snapper and Muise, {Aleixo M.} and Kappelman, {Michael D.}",

note = "Publisher Copyright: {\textcopyright} 2020 Crohn's & Colitis Foundation. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.",

year = "2021",

month = mar,

day = "1",

doi = "10.1093/ibd/izaa080",

language = "English (US)",

volume = "27",

pages = "295--302",

journal = "Inflammatory bowel diseases",

issn = "1078-0998",

publisher = "John Wiley and Sons Inc.",

number = "3",

}

TY - JOUR

T1 - Natural History of Very Early Onset Inflammatory Bowel Disease in North America

T2 - A Retrospective Cohort Study

AU - Kerur, Basavaraj

AU - Benchimol, Eric I.

AU - Fiedler, Karoline

AU - Stahl, Marisa

AU - Hyams, Jeffrey

AU - Stephens, Michael

AU - Lu, Ying

AU - Pfefferkorn, Marian

AU - Alkhouri, Raza

AU - Strople, Jennifer

AU - Kelsen, Judith

AU - Siebold, Leah

AU - Goyal, Alka

AU - Rosh, Joel R.

AU - Leleiko, Neal

AU - Van Limbergen, Johan

AU - Guerrerio, Anthony L.

AU - Maltz, Ross

AU - Karam, Lina

AU - Crowley, Eileen

AU - Griffiths, Anne

AU - Heyman, Melvin B.

AU - Deneau, Mark

AU - Benkov, Keith

AU - Noe, Joshua

AU - Mouton, Dedrick

AU - Pappa, Helen

AU - Galanko, Joseph A.

AU - Snapper, Scott

AU - Muise, Aleixo M.

AU - Kappelman, Michael D.

PY - 2021/3/1

Y1 - 2021/3/1

N2 - 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.

AB - 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.

KW - VEOIBD

KW - epidemiology

KW - surgery

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U2 - 10.1093/ibd/izaa080

DO - 10.1093/ibd/izaa080

M3 - Article

C2 - 32386060

AN - SCOPUS:85097773579

SN - 1078-0998

VL - 27

SP - 295

EP - 302

JO - Inflammatory bowel diseases

JF - Inflammatory bowel diseases

IS - 3

ER -

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

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