Invasive Pulmonary Fungal Infections in Cystic Fibrosis

Abstract

Invasive pulmonary mycosis is after allergic bronchopulmonary aspergillosis (ABPA) a frequent and severe complication of CF lung disease. Among CF caregivers, there is an insecurity when and how to treat infections of the lung parenchyma caused by different fungi in patients with CF. This case series provides a multicenter experience on diagnostic, manifestation, and treatment of non-ABPA cases of pulmonary. Non-ABPA cases of pulmonary mycoses in patients with CF have been collected from the CF Centers in Berlin, Essen, Worms, Frankfurt (Germany), Leeds (UK), and Barcelona (Spain). Non-ABPA was defined as total serum IgE level <500 kU/L. Scedosporium and Lomentospora species seem to be more virulent in patients with CF and have been successfully treated with triple antifungal drug regimens in several cases. Rare fungi including yeasts can have pathogenic potential in CF. In this series, antibiotic treatment failure was the main indicator for the initiation of antifungal treatment. For an early and effective treatment of pulmonary mycoses in CF, the identification of biomarkers and of risk factors beyond antibiotic treatment failure is crucial and urgently needed. Furthermore, treatment efficacy studies are necessary for the different causative agents of these infections.

The myriad challenges of respiratory fungal infection in cystic fibrosis

“…This class is generally well tolerated with similar rates of hepatotoxicity and infusion reactions across the three drugs. In cases of azole‐resistance authors have recommended combination therapy with an echinocandin in addition to triazoles …”

Fungal Respiratory Infections in Cystic Fibrosis (CF): Recent Progress and Future Research Agenda

“…The efficacy of antifungals drugs, used as single antifungal treatment or in combinations, is detailed along with new therapies and prevention strategies [6]. The third article in the clinical section is from another group led by Schwarz [7]. It provides details of the clinical management for eighteen cases of respiratory mycoses from six different CF centers of excellence.…”

“…It provides details of the clinical management for eighteen cases of respiratory mycoses from six different CF centers of excellence. The etiologic agents include Aspergillus species, Lomentospora prolificans, and other pathogenic fungi [7]. Many of the patients had clinical complications due to comorbidities, but good outcomes were obtained with the triple combination of antifungal drugs in nearly half of the cases.…”

“…Many of the patients had clinical complications due to comorbidities, but good outcomes were obtained with the triple combination of antifungal drugs in nearly half of the cases. The article by Schwarz et al [7] underscores the unmet needs for biomarkers to track fungal diseases and accurate antifungal laboratory tests to direct antifungal combination therapy.…”

“…Fungal diagnostic criteria in CF patients [6,9,12,13] 3. Antifungal therapy in CF patients [7,9,[14][15][16][17] 4. Aspergillus, Exophiala and Pneumocystis in CF patients [8][9][10][11] 5.…”