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Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab

Gastrointestinal sarcoidosis is a rare disease with very limited data in children. Here we report the first pediatric case of successful treatment with infliximab. The first case was an 8-year-old Saudi girl who presented with fever, weight loss, and abdominal pain that was followed in a few months...

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Autores principales: Alawdah, Laila, Nahari, Ahmad, Alshahrani, Dayel, Fagih, Musa, Ghazi, Shahid, Al-Hussaini, Abdulrahman
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5051225/
https://www.ncbi.nlm.nih.gov/pubmed/27748327
http://dx.doi.org/10.4103/1319-3767.191146
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author Alawdah, Laila
Nahari, Ahmad
Alshahrani, Dayel
Fagih, Musa
Ghazi, Shahid
Al-Hussaini, Abdulrahman
author_facet Alawdah, Laila
Nahari, Ahmad
Alshahrani, Dayel
Fagih, Musa
Ghazi, Shahid
Al-Hussaini, Abdulrahman
author_sort Alawdah, Laila
collection PubMed
description Gastrointestinal sarcoidosis is a rare disease with very limited data in children. Here we report the first pediatric case of successful treatment with infliximab. The first case was an 8-year-old Saudi girl who presented with fever, weight loss, and abdominal pain that was followed in a few months with hematemesis and development of hepatosplenomegaly. The second case was a 9-year-old Sudanese boy who manifested with vomiting, epigastric pain, and weight loss. On upper endoscopy, both cases demonstrated severe erosive nodular gastric mucosa. Gastric and esophageal biopsies had shown noncaseating granulomatous inflammation. The first case had histopathological evidence of granulomatous hepatitis, and both cases demonstrated lung nodularity on computed tomography chest. The boy had elevated angiotensin-converting enzyme level. Given the multisystem involvement with significant chest findings, tissue findings of granulomatous disease, and negative workup for other causes of granulomatous inflammation, both cases were diagnosed with active disseminated sarcoidosis, and treated with corticosteroids. The girl continued to be symptom-free for 4 years after tapering steroid therapy. The boy had relapses off steroids and the disease was brought into remission for 5 years off steroid therapy by infliximab. Pediatric GI sarcoidosis is a rare disease that exhibits heterogeneity in natural course. The chronic relapsing progressive form of the disease might benefit from infliximab therapy.
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spelling pubmed-50512252016-10-07 Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab Alawdah, Laila Nahari, Ahmad Alshahrani, Dayel Fagih, Musa Ghazi, Shahid Al-Hussaini, Abdulrahman Saudi J Gastroenterol Case Report Gastrointestinal sarcoidosis is a rare disease with very limited data in children. Here we report the first pediatric case of successful treatment with infliximab. The first case was an 8-year-old Saudi girl who presented with fever, weight loss, and abdominal pain that was followed in a few months with hematemesis and development of hepatosplenomegaly. The second case was a 9-year-old Sudanese boy who manifested with vomiting, epigastric pain, and weight loss. On upper endoscopy, both cases demonstrated severe erosive nodular gastric mucosa. Gastric and esophageal biopsies had shown noncaseating granulomatous inflammation. The first case had histopathological evidence of granulomatous hepatitis, and both cases demonstrated lung nodularity on computed tomography chest. The boy had elevated angiotensin-converting enzyme level. Given the multisystem involvement with significant chest findings, tissue findings of granulomatous disease, and negative workup for other causes of granulomatous inflammation, both cases were diagnosed with active disseminated sarcoidosis, and treated with corticosteroids. The girl continued to be symptom-free for 4 years after tapering steroid therapy. The boy had relapses off steroids and the disease was brought into remission for 5 years off steroid therapy by infliximab. Pediatric GI sarcoidosis is a rare disease that exhibits heterogeneity in natural course. The chronic relapsing progressive form of the disease might benefit from infliximab therapy. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC5051225/ /pubmed/27748327 http://dx.doi.org/10.4103/1319-3767.191146 Text en Copyright: © 2016 Saudi Journal of Gastroenterology (Official journal of The Saudi Gastroenterology Association) http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Case Report
Alawdah, Laila
Nahari, Ahmad
Alshahrani, Dayel
Fagih, Musa
Ghazi, Shahid
Al-Hussaini, Abdulrahman
Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab
title Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab
title_full Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab
title_fullStr Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab
title_full_unstemmed Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab
title_short Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab
title_sort pediatric gastrointestinal sarcoidosis: successful treatment with infliximab
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5051225/
https://www.ncbi.nlm.nih.gov/pubmed/27748327
http://dx.doi.org/10.4103/1319-3767.191146
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