Abstract
Defects in innate immune responses appear to predispose to inflammatory bowel disease (IBD) similar to Crohn's disease (CD), and 30% of patients with chronic granulomatous disease (CGD), a genetic disease of the innate immune system, develop IBD. Antibody panels are used to assist in the diagnosis of IBD in symptomatic patients. The aim of this study was to determine the usefulness of serologic testing for IBD in children with CGD. The medical records of 9 patients with CGD were reviewed for symptoms of IBD, and a serologic panel for IBD was performed. The panel measures ASCA IgA and IgG ELISA, Anti-Omp C IgA ELISA, Anti-CBir 1 ELISA, and IBD-specific pANCA. A proprietary formula is used to report IBD likely. All 9 patients had IBD serologic findings consistent with CD; nevertheless, only 2 of the 9 patients had IBD symptoms: diarrhea and mucoid stools in one, and mild diarrhea and growth failure in the other. Endoscopy performed in these 2 patients revealed granulomatous colitis in one individual only. These findings indicate that IBD serologic testing does not identify CGD patients with IBD. The consistently positive IBD panels in our CGD patients suggest that this innate immune deficiency predisposes to antigen sensitization that is seen in CD. However, other factors appear to determine which CGD patients progress to clinical granulomatous IBD.
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