Chronic granulomatous disease associated inflammatory bowel disease primarily involves the anus and rectum (SK Khangura et. al. Clin Gastroenterol Hepatol 2016; 14: 395-402). In this study from the NIH, among 78 patients with CGD-IBD enteric fisula were found in 18% (73% were perianal), colonic stictures were noted in 24% (80% in anorectal area).
- -EGD showed inflammation in 21%, 74%, and 37% of patients in esophageal, gastric and duodenal biopsies respectively
- -Small bowel disease was unusual, though two of the four patients with ileostomies had ileal ulcers
- -” ‘Target-like lesions,’ which have a clear elevated center with surrounding erythema, were characteristic of mild disease
- -Approximately 40% of CGD patients have GI tract involvement
- -GI tract malignancy was not observed in this cohort
- -24 patients had pancolitis
My take: This reference provides comprehensive details of the clinical features of a large cohort with CGD.