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UK: insights into practices in managing fungal lung disease in cystic fibrosis

A recent survey conducted in the United Kingdom has highlighted variability in diagnosis and management of respiratory tract fungal disease in patients with cystic fibrosis (CF). It also indicates a lack of sufficient evidence to guide management of fungal infection in CF.

Researchers conducted a survey with the aim of evaluating the current practices in diagnosis and management of fungal lung disease in patients with CF across the United Kingdom. 94 clinicians affiliated with recognised CF centres were invited to complete a 21-question anonymous online survey. The survey response rate was 60.6%.

98.2% of respondents reported using serum IgE for diagnosis of allergic bronchopulmonary aspergillosis (ABPA), whereas only 12.3% used skin prick testing and 14% used galactomannan testing.

For a first diagnosis of ABPA, 66.7% of respondents reported using prednisolone and itraconazole as the preferred treatment regimen, 35.1% reported using prednisolone alone and 3.5% chose prednisolone with voriconazole. For treating the first relapse, 58% used prednisolone with itraconazole, whereas 8.8% used prednisolone alone. For treatment reduction, 36.8% reported reducing steroids to zero over time and maintaining azole therapy, 31.6% said they would stop azole and steroid after a certain time and 8.8% reported stopping azole after a certain time and maintaining a small steroid dose.

66.7% believed that chronic colonisation of the airways by Aspergillus could result in clinical deterioration and 66.1% said they would treat this. 61.4% reported experience in treating patients with Scedosporium apiospermum, with voriconazole being the most common treatment of choice.

Writing in the journal Medical Mycology, the authors said "There are several challenges in CF respiratory tract fungal disease. We must rise to the challenge of understanding our treatment choices and establishing an evidence base for them."


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