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Invasive Pulmonary Fungal Infections in Cystic Fibrosis

Carsten Schwarz1, Claudia Brandt2, Paul Whitaker3

  • 1Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany. carsten.schwarz@charite.de.

Mycopathologia
|September 3, 2017
PubMed

Insights

Pulmonary mycoses, excluding allergic bronchopulmonary aspergillosis (ABPA), are serious in cystic fibrosis (CF). Early diagnosis and targeted antifungal treatment are vital for better outcomes in CF patients.

Area of Science:

  • Pulmonology
  • Infectious Diseases
  • Mycology

Background:

  • Invasive pulmonary mycosis is a frequent and severe complication in cystic fibrosis (CF) lung disease, often following allergic bronchopulmonary aspergillosis (ABPA).
  • Caregivers express uncertainty regarding the diagnosis and treatment of fungal lung infections in CF patients.
  • Non-ABPA pulmonary mycoses represent a significant challenge in CF management.

Purpose of the Study:

  • To present a multicenter experience on the diagnosis, manifestation, and treatment of non-ABPA pulmonary mycoses in patients with CF.
  • To highlight the role of specific fungal species and treatment strategies.
  • To identify needs for improved diagnostic and therapeutic approaches.

Main Methods:

  • A case series approach was employed, collecting data from CF centers in Germany, the UK, and Spain.
  • Non-ABPA was defined by a total serum IgE level below 500 kU/L.
  • Data on diagnostic methods, clinical manifestations, and treatment regimens were analyzed.

Main Results:

  • Scedosporium and Lomentospora species demonstrated higher virulence in CF patients.
  • Successful treatment of these infections was achieved with triple antifungal drug regimens in several instances.
  • Antibiotic treatment failure was a primary indicator for initiating antifungal therapy.

Conclusions:

  • Rare fungi, including yeasts, can be pathogenic in CF patients.
  • Identifying biomarkers and risk factors beyond antibiotic treatment failure is crucial for early and effective management of pulmonary mycoses in CF.
  • Further treatment efficacy studies are necessary for various causative agents.
Keywords:
Cystic fibrosisInvasive pulmonary mycosisNon-ABPA infectionsScedosporium

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