FUNGAL PULMONARY SYNDROMES IN CYSTIC FIBROSIS
ESPID Education. De Boeck K. 05/10/12; 20822
Prof. Kris De Boeck
Prof. Kris De Boeck

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Abstract
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In patients with cystic fibrosis (CF), lung infection with bacterial pathogens such as Staphylococcus aureus and Pseudomonas aeruginosa is the predominant problem. However fungal infections and complications involving fungal antigens are increasingly recognized. The best known fungal complication, occurring in 5 to 10 % of patients, is ‘allergic bronchopulmonary aspergillosis’ (ABPA). Patients present with a variety of signs and symptoms: increased cough and wheeze, new chest infiltrate, serum IgE> 500 IU/ml, and specific IgE and IgG antibodies against Aspergillus fumigatus. Diagnostic criteria are not clear-cut. Some patients definitely have the full blown picture and in others it is dubious whether they do have ABPA, a type of ‘Aspergillus astma’ or mere Aspergillus colonization. The treatment of ABPA is controversial, with oral corticosteroids and itraconazole as main stay therapy. Because of incomplete control and relapse, alternative therapies such as omalizumab, nebulized amphotericin B, pulse steroids and newer triazole agents are used. Worse evolution of lung function has been found in patients with persistent isolation of Aspergillus from sputum in the absence of ABPA. Whether antifungal treatment improves this course is unclear. Mainly in sicker and older patients, fungal pathogens such as Scedosporium, Exophiala and others are isolated. The treatment schedules for these are poorly researched. In CF patients, use of voriconazole is complicated by cost, complex drug-drug interactions and side effects. Posaconazole has greater activity against Aspergillus and causes fewer drug interactions. Experience in CF is however limited.
In patients with cystic fibrosis (CF), lung infection with bacterial pathogens such as Staphylococcus aureus and Pseudomonas aeruginosa is the predominant problem. However fungal infections and complications involving fungal antigens are increasingly recognized. The best known fungal complication, occurring in 5 to 10 % of patients, is ‘allergic bronchopulmonary aspergillosis’ (ABPA). Patients present with a variety of signs and symptoms: increased cough and wheeze, new chest infiltrate, serum IgE> 500 IU/ml, and specific IgE and IgG antibodies against Aspergillus fumigatus. Diagnostic criteria are not clear-cut. Some patients definitely have the full blown picture and in others it is dubious whether they do have ABPA, a type of ‘Aspergillus astma’ or mere Aspergillus colonization. The treatment of ABPA is controversial, with oral corticosteroids and itraconazole as main stay therapy. Because of incomplete control and relapse, alternative therapies such as omalizumab, nebulized amphotericin B, pulse steroids and newer triazole agents are used. Worse evolution of lung function has been found in patients with persistent isolation of Aspergillus from sputum in the absence of ABPA. Whether antifungal treatment improves this course is unclear. Mainly in sicker and older patients, fungal pathogens such as Scedosporium, Exophiala and others are isolated. The treatment schedules for these are poorly researched. In CF patients, use of voriconazole is complicated by cost, complex drug-drug interactions and side effects. Posaconazole has greater activity against Aspergillus and causes fewer drug interactions. Experience in CF is however limited.
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