Objectives: The aim of the study was to evaluate the prevalence of different variants of pulmonary aspergillosis in CF patients
Methods: In 2014-2017 yy. in prospective study were included 190 CF patients, 130 children. The median age was 14 y (range 1 - 37), 96 males. In all patients were performed total IgE test, specific A. fumigatusIgEand IgG tests in serum, and skin tests with six fungal allergens. In case of pulmonary aspergillosis suspicion chest CT scan was made, and bronchoalveolar lavage (BAL) and sputum were examined with calcofluor white microscopy and culture. The diagnosis of CPA was established with Denning et al. 2016 criteria, ABPA – Stevens et al. 2003, IA – EORCT/MCG 2008.
Results: Total IgE serum level varied from 1 to 3891 IU/ml (median - 149). Sensitization to fungi was identified in 57% patients. The highest frequency of fungal sensitization was connected with Candidaspp. (73%), Alternaria spp. (34%) andAspergillus spp. (27%). Occurrence of sensitization to other fungi: Rhizopusspp. – 20%, Penicilliumspp. – 10%, and Cladosporiumspp. – 6%.A. fumigatus IgG was detected in 68% patients. BAL and sputum cultures identified A. fumigatusin 18% patients, A.flavus- 4,7%,A. niger- 3,7%, A. nidulans – 0,5%, andA. tereus - 0,5%. ABPA was diagnosed in 4,7% patients, CPA– 4,2% patients, and invasive aspergillosis – 0,5%. Severe CF was observed in all patients with ABPA and CPA. Patient with IA underwent liver transplantation nine months before. The main clinical manifestations of ABPA were cough (100%) and dyspnea (100%), exacerbation of CF (22%) and fever (22%). In all patients with ABPA was identified sensitization to A. fumigatus, total serum IgE level varied from 196 to 2000 IU/ml (median – 990).Main etiology agents of ABPA were A. fumigatus (77%) andA. flavus (11%). In ABPA patients chest CT scan abnormalities were central bronchoectases (100%), infiltrates (100%) and atelectasis (11%). In patients with CPA the main clinical manifestations were exacerbation of CF (100%), cough (100%), fever (62%), and hemoptysis (50%). Low lung function (FEV1% <70%) was in 5 (63%) patients. A. fumigatusIgG test was positive in all patients with CPA. Etiology agentsof CPA: A. fumigatus(86%)and A. niger(14%). Chest CT scan abnormalities were central bronchoectases (100%), infiltrates and focal changes (88%), and aspergilloma (11%). All patients received antifungal treatment. In ABPA patients voriconazole was used in 66%, itraconazole – 33%. Median duration of antifungal treatment was 132 days (42-390), in 44% cases we observed ABPA relapse. In CPA patients voriconazole was used in 87%, posaconazole – 25%, median duration of antifungal treatment was 86 days (14-210). Patient with IA was successfully treated with voriconazole for 12 months. Overall survival in 12 months in ABPA patients was 100%, CPA patients – 87%, IA – 100%. 
Conclusion: The frequency of different forms of aspergillosis in cystic Fibrosis patients was 9,4% (ABPA - 4,7%, CPA– 4,2%, and invasive aspergillosis – 0,5%).

Aspergillosis in Cystic Fibrosis Patients in Russia – the results of a prospective research