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Case Reports
. 2022 Jan 24;3(1):e169.
doi: 10.1097/PG9.0000000000000169. eCollection 2022 Feb.

Dual Biologic Therapy in an Adolescent With Camurati-Engelmann Disease and Crohn Disease

Ahmad Salah Sami  1 , Joel R Rosh  2
Affiliations
Case Reports

Dual Biologic Therapy in an Adolescent With Camurati-Engelmann Disease and Crohn Disease

Ahmad Salah Sami et al. JPGN Rep. .

Abstract

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.

Keywords: TGF-β1; inflammatory bowel disease; ustekinumab; vedolizumab.

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Conflict of interest statement

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.

Figures

FIGURE 1.
FIGURE 1.
Albumin, hemoglobin, and C-reactive protein levels before and after initiating dual biologic therapy (Vedo: Vedolizumab; Ustek: Ustekinumab).
FIGURE 2.
FIGURE 2.
Recorded BMI levels before and after initiating dual biologic therapy (Vedo: Vedolizumab; Ustek: Ustekinumab). BMI, body mass index.
See this image and copyright information in PMC

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