Inflammatory Bowel Disease in Chronic Granulomatous Disease: an emerging problem over a twenty years' experience.

Inflammatory Bowel Disease in Chronic Granulomatous Disease: an emerging problem over a twenty years' experience.

Pediatr Allergy Immunol. 2017 Oct 05;:

Authors: Angelino G, De Angelis P, Faraci S, Rea F, Romeo EF, Torroni F, Tambucci R, Claps A, Francalanci P, Chiriaco M, Di Matteo G, Cancrini C, Palma P, D'Argenio P, Dall'Oglio L, Rossi P, Finocchi A

Abstract
BACKGROUND: Chronic Granulomatous Disease (CGD) is a primary immunodeficiency of phagocytes, characterized by life-threatening infections and hyperinflammation. Due to survival improvement, Inflammatory Bowel Disease (IBD) is becoming increasingly relevant. Here we report our 20-year experience.
METHODS: We retrospectively analyzed clinical, endoscopic and histologic features, as well as the management of CGD-IBD patients referred to the Bambino Gesù Children's Hospital in Rome, Italy.
RESULTS: Out of 20 CGD patients, 9 presented with CGD-IBD at diagnosis and/or during follow-up. Symptoms occurred at a median age of 16 years (range 3.2-42), with a median delay of 6 months for endoscopic confirmation. Patients mainly complained of nonspecific diarrhea (55%), with discrepancy between symptom paucity and severe endoscopic appearance, mainly represented by extensive colonic involvement (44%). Histology revealed at least 2 characteristic features (epithelioid granulomas, pigmented macrophages, and increased eosinophils) in 78% of patients. Eight out of 9 patients received oral mesalamine and 5 required systemic steroids. One patient received azathioprine due to steroid dependence. No patient required biological therapy, or surgery. Clinical remission was obtained in all patients, but the majority complained of mild relapses. Two episodes of severe infection occurred early after steroid therapy.
CONCLUSIONS: Penetrance of CGD-IBD increases with age. Clinical manifestations may be subtle, and clinicians should have a low threshold to recommend endoscopy. Treatment with NSAIDs and/or steroids achieves a good response, but relapses usually occur. Infection surveillance is mandatory during treatment, in order to prevent opportunistic infections. A close collaboration between Immunologists and Gastroenterologists is pivotal, including combined follow-up. This article is protected by copyright. All rights reserved.

PMID: 28981976 [PubMed - as supplied by publisher]

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