Publication Date
2018
Journal Title
J Pediatric Infect Dis Soc
Abstract
© The Author(s) 2018. Chronic granulomatous disease (CGD), a primary immunodeficiency characterized by a deficient neutrophil oxidative burst and the inadequate killing of microbes, is well known to cause a significantly increased risk of invasive infection. However, infectious complications are not the sole manifestations of CGD; substantial additional morbidity is driven by noninfectious complications also. These complications can include, for example, a wide range of inflammatory diseases that affect the gastrointestinal tract, lung, skin, and genitourinary tract and overt autoimmune disease. These diseases can occur at any age and are especially problematic in adolescents and adults with CGD. Many of these noninfectious complications present a highly challenging therapeutic conundrum, wherein immunosuppression must be balanced against an already markedly increased risk of invasive fungal and bacterial infections. In this review, the myriad noninfectious complications of CGD are discussed, as are important gaps in our understanding of these processes, which warrant further investigation.
Volume Number
7
Pages
S18 - S24
Document Type
Article
Status
Faculty
Facility
School of Medicine
Primary Department
Allergy and Immunology
Additional Departments
General Pediatrics
PMID
DOI
10.1093/jpids/piy014