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Patients with Primary Immunodeficiencies: How Are They at Risk for Fungal Disease?

  • Current Management of Fungal Infections (J Maertens, Section Editor)
  • Published:
Current Fungal Infection Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

In this review, we focus on the inborn errors of immunity known to render the host susceptible to fungal infections, including candidias, aspergillosis, dermatophytosis, phaeohyphomycosis, pneumocystosis, fusariosis, cryptococcosis, and endemic mycoses.

Recent Findings

Classically, the burden of fungal disease in humans is believed to be carried by patients with a secondary immunodeficiency, either due to malignancy, to chemotherapy, to an immunocompromised state post hematopoietic stem cell transplantation, or to treatment with anti-cytokine therapies. However, in the last decade, the study of patients affected by fungal infections without any overt risk factors has led to the unraveling of several monogenic defects of human immunity to fungi. The study of these inborn errors of immunity has added vastly to our comprehension of antifungal immunity. For example, the role of IL-17 immunity in human defense against mucocutaneous candidiasis has been extensively characterized through the analysis of IL-17F, IL-17RA, IL-17Rc, ACT1, RORγT and, indirectly, CARD9 deficiency.

Summary

Many monogenic causes of susceptibility to superficial and/or invasive fungal infections have been recently unraveled. Most of these inborn errors of immunity associate with a specific type of fungal infection, and such a defect should always be suspected and sought in patients affected by fungal infection in the absence of predisposing factors.

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Authors and Affiliations

  1. Department of Immunology and Microbiology, Laboratory of Inborn Errors of Immunity, KU Leuven, Leuven, Belgium

    Giorgia Bucciol, Leen Moens & Isabelle Meyts

  2. Department of Pediatrics, Laboratory of Inborn Errors of Immunity, University Hospitals Leuven, Herestraat 49, 3000, Leuven, Belgium

    Giorgia Bucciol & Isabelle Meyts

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  1. Giorgia Bucciol
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  2. Leen Moens
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  3. Isabelle Meyts
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Correspondence to Isabelle Meyts.

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This article is part of the Topical Collection on Current Management of Fungal Infections

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Bucciol, G., Moens, L. & Meyts, I. Patients with Primary Immunodeficiencies: How Are They at Risk for Fungal Disease?. Curr Fungal Infect Rep 12, 170–178 (2018). https://doi.org/10.1007/s12281-018-0323-z

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