Background: ABPA is a rare complication of cystic fibrosis (CF) caused by allergic inflammation of the lower respiratory tract due to sensitization to Aspergillus fumigatus. Standard therapeutic options include systemic corticosteroids and antimycotics such as itraconazole. In 2009 we could demonstrate that ABPA does not necessarily lead to an additional decrease in lung function. Whether there are significant side effects to the therapy remains unclear. Methods: During 12 years we collected data from 26 ABPA patients (Stevens' criteria CID, 2003) in a pair controlled restrospective study. Patients were matched for age, gender, pseudomonas infection status and lung function. All patients were treated with corticosteroids (initially 0,5-2mg/kg) and itraconazole. Results: 15 patients (9 girls, 6 boys, mean age 7,2 Jahre, mean FEV1 91,9% before diagnosis, 12 of them with chronic pseudomonas infection were followed up over 5 years. The decrease in FEV1 in the ABPA group was not significantly different from the controls, but there was significant growth retardation in the ABPA group (mean deterioration of growth percentiles/year: -2,6 vs -0,7%, p = 0,33) There was no difference in the prevalence of diabetes in both groups, but an increased number of ABPA patients developed impaired glucose tolerance during the follow up period (27 vs 0%, p = 0,039). Conclusion: Timely and aggressive steroid therapy of ABPA protects from significant decrease in lung function in comparison to a matched control group. Side effects of this treatment are severe and include significant growth retardation and increased risk of impaired glucose tolerance.
Full conference title:
20th European Respiratory Society conference
- ERS 20th (2010)