Dual Biologic Therapy in an Adolescent With Camurati-Engelmann Disease and Crohn Disease.
TGF-β1
inflammatory bowel disease
ustekinumab
vedolizumab
Journal
JPGN reports
ISSN: 2691-171X
Titre abrégé: JPGN Rep
Pays: United States
ID NLM: 101773885
Informations de publication
Date de publication:
Feb 2022
Feb 2022
Historique:
received:
10
01
2021
accepted:
08
12
2021
medline:
24
1
2022
pubmed:
24
1
2022
entrez:
11
5
2023
Statut:
epublish
Résumé
Camurati-Engelmann disease (CED) is a rare disorder caused by activating mutations in the TGF-β1 gene and characterized by hyperostosis of long bones and bone dysplasia. We describe a case of an adolescent with CED and moderate-severe Crohn Disease (CD). Infliximab improved gastrointestinal symptoms but was associated with worsening CED-associated bone pain. Clinical remission was successfully achieved with dual biologic therapy that included vedolizumab and ustekinumab. Possible reasons for this patient's clinical response are advanced and include speculation about the complex role of TGF-β1 signaling in the etiology of both CED and CD.
Identifiants
pubmed: 37168741
doi: 10.1097/PG9.0000000000000169
pmc: PMC10158315
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e169Informations de copyright
Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
Déclaration de conflit d'intérêts
A.S.S. report no conflicts of interest. J.R.R. received grant/research from Abbvie, Janssen; and advisor/consultant from BMS, Celgene, Janssen, Lilly, Pfizer.
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