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Scientific article
Review
Open access
English

Hyperinflammation in chronic granulomatous disease and anti-inflammatory role of the phagocyte NADPH oxidase

Published inSeminars in immunopathology, vol. 30, no. 3, p. 255-271
Collection
  • Open Access - Licence nationale Springer
Publication date2008
Abstract

Chronic granulomatous disease (CGD) is an immunodeficiency caused by the lack of the superoxide-producing phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. However, CGD patients not only suffer from recurrent infections, but also present with inflammatory, non-infectious conditions. Among the latter, granulomas figure prominently, which gave the name to the disease, and colitis, which is frequent and leads to a substantial morbidity. In this paper, we systematically review the inflammatory lesions in different organs of CGD patients and compare them to observations in CGD mouse models. In addition to the more classical inflammatory lesions, CGD patients and their relatives have increased frequency of autoimmune diseases, and CGD mice are arthritis-prone. Possible mechanisms involved in CGD hyperinflammation include decreased degradation of phagocytosed material, redox-dependent termination of proinflammatory mediators and/or signaling, as well as redox-dependent cross-talk between phagocytes and lymphocytes (e.g. defective tryptophan catabolism). As a conclusion from this review, we propose the existence of ROS high and ROS low inflammatory responses, which are triggered as a function of the level of reactive oxygen species and have specific characteristics in terms of physiology and pathophysiology.

Keywords
  • Animals
  • Granulomatous Disease, Chronic/enzymology/immunology/pathology
  • Humans
  • Inflammation/enzymology
  • Mice
  • NADPH Oxidase/immunology
Citation (ISO format)
SCHAEPPI, Michela et al. Hyperinflammation in chronic granulomatous disease and anti-inflammatory role of the phagocyte NADPH oxidase. In: Seminars in immunopathology, 2008, vol. 30, n° 3, p. 255–271. doi: 10.1007/s00281-008-0119-2
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Article (Published version)
accessLevelPublic
Identifiers
ISSN of the journal1863-2297
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307downloads

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