Noninfectious Manifestations and Complications of Chronic Granulomatous Disease
Open Access
- 9 May 2018
- journal article
- research article
- Published by Oxford University Press (OUP) in Journal of the Pediatric Infectious Diseases Society
- Vol. 7 (suppl_1), S18-S24
- https://doi.org/10.1093/jpids/piy014
Abstract
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.This publication has 82 references indexed in Scilit:
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