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Seminars in Immunopathology
Published in: Seminars in Immunopathology 3/2008

01-07-2008 | Review

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

Authors: Michela G. Schäppi, Vincent Jaquet, Dominique C. Belli, Karl-Heinz Krause

Published in: Seminars in Immunopathology | Issue 3/2008

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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 ROShigh and ROSlow 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.
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Metadata
Title
Hyperinflammation in chronic granulomatous disease and anti-inflammatory role of the phagocyte NADPH oxidase
Authors
Michela G. Schäppi
Vincent Jaquet
Dominique C. Belli
Karl-Heinz Krause
Publication date
01-07-2008
Publisher
Springer-Verlag
Published in
Seminars in Immunopathology / Issue 3/2008
Print ISSN: 1863-2297
Electronic ISSN: 1863-2300
DOI
https://doi.org/10.1007/s00281-008-0119-2

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