Fungal infections in primary immunodeficiency diseases

Clin Immunol. 2020 Oct;219:108553. doi: 10.1016/j.clim.2020.108553. Epub 2020 Jul 30.


Primary immunodeficiency diseases (PID), encompass a heterogeneous group of diseases, with increased susceptibility to recurrent, severe infections. Invasive fungal infections raise a serious concern related to their morbidity and mortality. Herein, we describe various fungal infections among different PID patients. Twenty-eight PID patients diagnosed with fungal infections were included; fourteen patients with chronic granulomatous disease, two with Hyper Immunoglobulin E syndrome, one with LRBA deficiency and one with MHC class II defect, one with unclassified immune dysregulation, one with CD4 lymphopenia and one patient with Immune dysregulation Polyendocrinopathy Enteropathy X-linked syndrome. Aspergillus species were the most common isolated causative organisms in 78% of patients, Candida species were the causative organisms in 32%, Pneumocystis jirovecii caused infections in 7% followed by Malassezia furfur, Fusarium spp., Mucormycosis, and Penicillium chrysogenium 3.5% for each. The mortality rate among our patients was 10/28 (35.7%). PID patients are at high risk of developing fungal infections.

Keywords: Aspergillus infections; Candida infections; Fungal infections; Immunodeficiency diseases.

Publication types

  • Case Reports

MeSH terms

  • Bacteria / isolation & purification
  • Bacterial Infections / etiology
  • Bacterial Infections / microbiology
  • Child
  • Child, Preschool
  • Fatal Outcome
  • Female
  • Fungi / isolation & purification
  • Humans
  • Infant
  • Male
  • Mycoses / etiology*
  • Mycoses / microbiology
  • Primary Immunodeficiency Diseases / complications*
  • Primary Immunodeficiency Diseases / microbiology