Aspergillus fumigatus is commonly found in the airways of patients with Cystic Fibrosis, (CF). Allergic Bronchopulmonary Aspergillosis, (ABPA), is the most recognised clinical condition associated with Aspergillus. The most widely used diagnostic criteria are from the Cystic Fibrosis Foundation Consensus Conference 2003. However, diagnosis remains challenging due to the overlap of classical symptoms and radiological features of ABPA and CF. There are a lack of clinical trials with clear outcomes to guide management of fungal disease, leading to variability between CF centres.
The aim of this survey was to assess the variability in current practice across the UK in diagnosis and management of fungal lung disease in CF patients.
A 21 question anonymous online survey was sent to 94 paediatric and adult CF consultants in the UK.
The response rate was 60.6% with 55 full and 2 partially completed surveys. Thirty-two respondents were adult physicians and twenty-five paediatricians. For a first diagnosis of ABPA 20 (35.1%) treat with Prednisolone alone, 19 (33.3%) use Prednisolone with Itraconazole capsules, 19 (33.3%) use Prednisolone with Itraconazole liquid and 2 (3.5%) choose Voriconazole.
Only 5 (8.8%) treat with Prednisolone alone for a 1st relapse, preferring Prednisolone with Itraconazole Liquid (33.3%) or with Itraconazole capsules (24.6%).
To reduce treatment, 21 (36.8%) decrease steroids to zero over time and maintain azole therapy, 18 (31.6%) stop the azole and steroid after a fixed time and 5 (8.8%) stop azole after a fixed time and maintain a small steroid dose. Variations in specific therapies were reported, including the use of pulsed Methylprednisolone, Posaconazole, nebulised Amphotericin and Omalizumab.
Thirty-eight (66.7%) respondents believe Aspergillus colonisation of the airway can cause clinical deterioration and 37 (66.1%) would treat this. Scedosporium apiospermum infection has been diagnosed and treated by 35 (61.4%) of respondents.
Results of this survey highlight significant differences in treatment regimes for ABPA, with increasing variation seen in the management of subsequent relapses. Respondent comments showed a wide range of opinions. This survey highlights the lack of evidence currently available to guide the management of CF fungal disease.