Prevention of drug-related complications in cystic fibrosis

Meer, Renske van der; Touw, Daan; Heijerman, H.G.M.

doi:10.1097/mcp.0000000000000625

Cited by 2 publications

(2 citation statements)

References 82 publications

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“…hepatotoxicity, peripheral neuropathy), an increasing rate of A. fumigatus -resistant strains, and potential interactions with other drugs that are metabolised by hepatic cytochrome P450 [ 10 , 55 ]. Notably, by acting as a cytochrome P450 inhibitor, itraconazole, as with the other triazoles, may determine an increase in the serum concentrations of CFTR modulators; thus, drug monitoring and dose adaptation is required to avoid toxicity [ 56 ].…”

Section: Discussionmentioning

confidence: 99%

Biologic drugs in treating allergic bronchopulmonary aspergillosis in patients with cystic fibrosis: a systematic review

et al. 2022

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BackgroundAspergillus fumigatus is a common saprophytic fungus causing allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF). The recommended first-line treatment for ABPA is oral steroids, followed by antifungal therapy. However, both treatments are not free from adverse effects; thus, efforts are being made to identify new drugs showing the same effectiveness but with fewer or no side-effects. Therein, biologic drugs have been significantly implemented in clinical practice in treating ABPA in patients with CF.ObjectiveTo systematically review the available literature, providing evidence for the administration of biologic drugs as a new potential treatment of ABPA in both the paediatric and adult populations with CF.MethodsA systematic review of the literature published between January 2007 and July 2021 was performed, using a protocol registered with the International Prospective Register of Systematic Reviews (PROSPERO CRD42021270932).ResultsA total of 21 studies focusing on the use of biologics in treating ABPA in CF patients was included. We highlighted a paucity of data providing evidence for biologic drug use in ABPA.ConclusionScientific evidence is insufficient to support firm conclusions and randomised clinical trials are urgently required to investigate the efficacy and safety of biologics for ABPA in CF patients.

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Section: Discussionmentioning

confidence: 99%

Biologic drugs in treating allergic bronchopulmonary aspergillosis in patients with cystic fibrosis: a systematic review

et al. 2022

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“…This has relevance for management of CF patients, as antifungal medications can have significant adverse effects, including hepatotoxicity, gastrointestinal disturbance and QTc prolongation [14]. They also interact with many common drugs and with CFTR modulators, necessitating dose adjustment [15]. Routine targeting of E. dermatitidis with antifungals for acute exacerbations may therefore cause harm and is unlikely to be of benefit.…”

Section: Discussionmentioning

confidence: 99%

Isolation of Exophiala dermatitidis is not associated with worse clinical outcomes during acute pulmonary exacerbations in cystic fibrosis

Tewkesbury

Looi

Barry

et al. 2022

Journal of Medical Microbiology

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Introduction. The black yeast Exophiala dermatitidis has been isolated in respiratory samples from people with cystic fibrosis (CF). However, adequate detection may require longer incubation periods than the current UK national standard for CF respiratory samples. Furthermore, it is unclear whether isolation of E. dermatitidis is associated with poorer clinical outcomes in CF. Hypothesis/gap statement. E. dermatitidis does not cause clinically significant lung disease in CF adults. Aim. To evaluate differences in clinical outcomes over a 12 month period and during acute pulmonary exacerbations between CF adults with and without untreated E. dermatitidis. Methodology. Incubation times for respiratory samples on Sabouraud dextrose agar with chloramphenicol (SABC) plates at a large regional adult CF centre were extended from 2 to 7 days over a 1 month period. The number of patients from whom E. dermatitidis was isolated, and the length of incubation time prior to isolation, were recorded. Outcomes of treatment of exacerbations with intravenous antibiotics but in the absence of concomitant antifungal therapy were compared between those with and without E. dermatitidis, as were changes in lung function and body mass index (BMI) over a 12 month period. Results. Extended incubation unmasked the presence of E. dermatitidis in 22 of 132 patients; all isolations occurred after >48 h of incubation. Patients who isolated E. dermatitidis had lower rates of Pseudomonas aeruginosa isolation (P=0.02) and higher rates of non-tuberculous mycobacteria isolation (P=0.03), and were more likely to be prescribed a long-term antifungal medication (P=0.03), but had no differences in age, sex, baseline lung function or body mass index (BMI). There were no differences in response to treatment of acute exacerbations between patients with and without E. dermatitidis, or in change in forced expiratory volume in 1 s (FEV1), BMI and number of exacerbations over 12 months of follow-up. Conclusion. E. dermatitidis is not associated with worse clinical outcomes in CF. Given potential side effects and drug interactions, routine targeting of E. dermatitidis with antifungals during acute exacerbations is not advised.

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Prevention of drug-related complications in cystic fibrosis

Cited by 2 publications

References 82 publications

Biologic drugs in treating allergic bronchopulmonary aspergillosis in patients with cystic fibrosis: a systematic review

Biologic drugs in treating allergic bronchopulmonary aspergillosis in patients with cystic fibrosis: a systematic review

Isolation of Exophiala dermatitidis is not associated with worse clinical outcomes during acute pulmonary exacerbations in cystic fibrosis

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