Clinician variability in the diagnosis and treatment of aspergillus fumigatus-related conditions in cystic fibrosis: An international survey

Gina Hong; Sameer Desai; Richard B Moss; Patience Eschenhagen; Bradley S Quon; Carsten Schwarz

doi:10.1016/j.jcf.2021.07.008

Clinician variability in the diagnosis and treatment of aspergillus fumigatus-related conditions in cystic fibrosis: An international survey

J Cyst Fibros. 2022 Jan;21(1):136-142. doi: 10.1016/j.jcf.2021.07.008. Epub 2021 Jul 29.

Authors

Gina Hong¹, Sameer Desai², Richard B Moss³, Patience Eschenhagen⁴, Bradley S Quon⁵, Carsten Schwarz⁴

Affiliations

¹ Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA. Electronic address: gina.hong@pennmedicine.upenn.edu.
² School of Population and Public Health, University of British Columbia, Vancouver, BC, Canada.
³ Center of Excellence in Pulmonary Biology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA.
⁴ Department of Pediatric Pneumology, Immunology and Intensive Care Medicine, CF Center, Charité-Universitätsmedizin Berlin, Berlin, Germany.
⁵ Department of Pediatric Pneumology, Immunology and Intensive Care Medicine, CF Center, Charité-Universitätsmedizin Berlin, Berlin, Germany; Centre for Heart Lung Innovation, University of British Columbia and St. Paul's Hospital; Division of Respiratory Medicine, Department of Medicine, University of British Columbia, Vancouver, BC, Canada.

Abstract

Background: The diagnosis and treatment of Aspergillus fumigatus (Af)-related conditions remain a challenge in cystic fibrosis (CF) due to overlapping features of disease and absence of clinical guidelines for Af-related conditions outside of ABPA.

Objective: To investigate the differences of clinical practice in the diagnosis and management of Af-related conditions in CF.

Methods: We conducted an international survey to CF clinicians to ascertain the screening, diagnostic, and treatment practices for Af-related conditions in CF. Respondents were grouped into geographical regions and regional comparisons using chi-square tests of independence or Fisher's tests were performed.

Results: A total of 319 survey responses from 35 countries were analyzed. We observed differences in use and frequency of fungus culture, Aspergillus-specific IgE and IgG, skin prick testing, and pulmonary function testing as screening for Af-related conditions between the geographical regions. ABPA and Aspergillus bronchitis diagnostic criteria selection differed by region; significantly greater proportion of United States (US) and Canadian clinicians were unable to define Aspergillus bronchitis compared to Europe and other regions. Decision to treat ABPA was uniform across regions, but the consideration of Aspergillus bronchitis as a clinical disease warranting therapy differed between regions. The use of glucocorticoid and itraconazole was the first-line treatment of ABPA among clinicians; however, prednisone monotherapy was more common in US and Canada.

Conclusions: Significant variability in the diagnosis and management of Aspergillus-related conditions in CF was observed. Future studies are necessary to better harmonize the approach to Af-related disease in CF.

Keywords: Allergic bronchopulmonary aspergillosis; Aspergillus bronchitis; Aspergillus colonization; Fungi.

Publication types

Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

MeSH terms

Aspergillosis / diagnosis*
Aspergillosis / therapy*
Aspergillus fumigatus
Cystic Fibrosis / microbiology*
Cystic Fibrosis / therapy*
Humans
Practice Patterns, Physicians'*
Surveys and Questionnaires

Grants and funding

K23 HL146970/HL/NHLBI NIH HHS/United States