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Chronic Granulomatous Disease and Aspergillosis

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Immunogenetics of Fungal Diseases

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Abstract

The phagocyte NADPH oxidase is rapidly activated as an emergency response to infection. Activation of the NADPH oxidase requires translocation of cytoplasmic constituents to a membrane-bound flavocytochrome, and results in the conversion of molecular oxygen to superoxide anion and downstream reactive oxidant metabolites. Chronic granulomatous disease (CGD) is an inherited disorder of the NADPH oxidase characterized by recurrent and severe bacterial and fungal infections. Invasive aspergillosis and other filamentous fungi are the major causes of mortality in CGD. CGD is also characterized by inflammatory disorders, including inflammatory bowel disease, obstructive granulomata of the genitourinary tract, and pneumonitis. These findings underscore the dual role of NADPH oxidase as a critical component of host defense and as a modulator of inflammation. Management of CGD patients involves antibacterial and antifungal prophylaxis, recombinant interferon-γ, and early diagnosis and treatment of infections. Hematopoietic stem cell transplantation is a potentially curative treatment for CGD, and is being used with greater frequency. Knowledge gained from CGD patients and engineered mouse models have resulted in important insight regarding host and fungal pathogen interactions that determine control versus progression of infection and pathways that activate and limit inflammatory responses. This knowledge is broadly important to our understanding of innate immunity and for the development of novel immunotherapy approaches.

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Authors and Affiliations

  1. Department of Medicine, Roswell Park Cancer Institute, Buffalo, NY, USA

    Melissa J. Grimm PhD

  2. Departments of Medicine and Immunology, Roswell Park Cancer Institute, and University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, USA

    Brahm H. Segal MD, FACP, FIDSA

  3. Division of Infectious Diseases, Department of Immunology, Roswell Park Cancer Institute, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, Elm & Carlton Streets, Buffalo, NY, 14263, USA

    Brahm H. Segal MD, FACP, FIDSA

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  1. Melissa J. Grimm PhD
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  2. Brahm H. Segal MD, FACP, FIDSA
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Correspondence to Brahm H. Segal MD, FACP, FIDSA .

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  1. Life and Health Sciences Research Institute (ICVS), University of Minho, School of Medicine, Braga, Portugal

    Agostinho Carvalho

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Grimm, M.J., Segal, B.H. (2017). Chronic Granulomatous Disease and Aspergillosis. In: Carvalho, A. (eds) Immunogenetics of Fungal Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-50842-9_4

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