Gastrointestinal complications in chronic granulomatous disease

Abstract

Almost half of patients with chronic granulomatous disease (CGD) suffer from gastrointestinal (GI) inflammation, the pathogenesis of which is complex and multifactorial. As a result, the management of CGD-associated GI inflammation remains challenging due to its chronicity and difficulty in managing the simultaneous need for immunomodulation with increased susceptibility to infection. In order to contextualize prospective treatment interventions for CGD-associated GI inflammation, we have reviewed the clinical presentation, pathogenesis and current management of this disease. Increased understanding of the role of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex 2 (NOX2)-derived reactive oxygen species (ROS) in inflammatory bowel disease (IBD) will likely reveal novel targets for therapeutic intervention.