The diagnosis and management of respiratory tract fungal infection in cystic fibrosis: A UK survey of current practice

Abstract

Aspergillus fumigatus is commonly found in the airways of patients with cystic fibrosis (CF), and allergic bronchopulmonary aspergillosis (ABPA) is the most recognized associated clinical condition. However, accurate diagnosis remains challenging, and there is a paucity of clinical trials to guide clinical management of fungal disease. 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% (32 adult physicians, 25 pediatricians) with 55 full and 2 partially completed surveys. For a first diagnosis of ABPA 20 (35.1%) treat with prednisolone alone, 38 (66.7%) use prednisolone with itraconazole and 2 (3.5%) choose voriconazole. Only 5 (8.8%) treat with prednisolone alone for a 1st relapse, 33 (58%) used prednisolone with itraconazole. 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 the azole after a fixed time and maintain a small steroid dose. Thirty-eight (66.7%) respondents believe Aspergillus colonization 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 the variance in clinical practice and the limited evidence available to guide management of fungal infection in CF.

Respiratory Fungal Diseases in Adult Patients With Cystic Fibrosis

“…49 Considering the reported low susceptibility of S. apiospermium complex to polyenes, azoles are usually employed as first-line therapy. 50…”

Clinical relevance of Scedosporium spp. and Exophiala dermatitidis in patients with cystic fibrosis: A nationwide study

“…The clinical relevance of these two filamentous fungi is unclear, leading to a lack of guidelines for clinical management. 16 Besides A. fumigatus , other filamentous fungi are increasingly reported as well, although mostly in small numbers and in single center studies. 13 , 15 , 17 Whether presence of Scedosporium spp.…”

“…and E. dermatitidis in the lungs only causes harmless colonization of the airways, or also constitutes a clinically relevant risk for patients with CF remains a matter of debate. 10 , 16 , 18 , 19 …”

Coinfection with Pseudomonas aeruginosa and Aspergillus fumigatus in cystic fibrosis

“…Diagnostic criteria for ABPA were established in 2003 [27], and are based on a range of clinical, serologic and radiological parameters that continue to be in widespread use today. While there is some variation in management practices, there is consensus amongst clinicians that a diagnosis of ABPA has significant clinical implications that warrants treatment with systemic corticosteroids, with or without antifungal therapy [51,52]. In addition to ABPA, Aspergillus-related lung disease may have other manifestations in people with CF.…”

“…However, this was a low-quality study comparing groups of patients from different time-points with variable rates of ABPA between the two cohorts . Even within ABPA, the role of antifungal therapy is unclear with variations in clinical practice amongst clinicians [52]. A systematic review of antifungal treatment in ABPA including four randomised controlled trials, showed that symptoms, frequency of pulmonary exacerbations and lung function all improved with antifungal treatment.…”