Allergic bronchopulmonary aspergillosis as a poor prognostic factor in cystic fibrosis patients with atelectasis

Pulmonary atelectasis is the least studied pulmonary complication in cystic fibrosis (CF). The following objectives have been proposed: 1) to determine whether allergic bronchopulmonary aspergillosis (ABPA) acts as a factor associated with a poor prognosis in patients who subsequently developed atelectasis (decline in lung function, exacerbations, transplantation, lung surgery and death), 2) to identify factors associated with the onset of APBA prior to the diagnosis of atelectasis, 3) therapeutic management, and 4) the clinical and radiological course of both conditions in combination.

A longitudinal, retrospective, multicentre study involving 14 national cystic fibrosis (CF) specialist units, including cases of persistent atelectasis and a minimum follow-up of two years. Data were collected on anthropometric measurements, genetics, imaging tests, placement on the transplant list, lung transplantation/surgery or death, extrapulmonary comorbidities, other pulmonary complications (infectious or non-infectious), lung function closest to the onset of atelectasis (baseline) and the most recent recorded value, number of mild-to-moderate or severe exacerbations in the year prior to and following the atelectasis and in the final year of follow-up, medical treatment, indications for flexible bronchoscopy and subsequent course.

55 patients with atelectasis (3.9%; 95% CI=3-5.1) were included, with a mean age of 25±10 years. Thirteen patients had ABPA (23.9%). Patients with ABPA had a higher number of mild-to-moderate exacerbations in the year prior to developing atelectasis, as well as in the final year of follow-up; and a higher total number of exacerbations in the year before and after the complication.

Patients with atelectasis who had previously presented with ABPA had a poorer prognosis due to an increase in exacerbations.