Presentation and diagnosis of allergic bronchopulmonary aspergillosis in cystic fibrosis

Early identification and management of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF) is critical, because ABPA can cause significant and rapid structural changes in the lungs. Approximately 10% of patients with CF have coexisting ABPA. Patients with CF have annual serological screening for ABPA, but clinicians should also be alert to signs of ABPA in between screenings.

In patients with CF, ABPA usually presents as acute or subacute clinical deterioration (eg wheezing, decreased exercise tolerance and reduced lung function). Differentiating between ABPA and a CF exacerbation can be difficult; see exacerbations of cystic fibrosis lung disease. ABPA may be associated with a dryer or ‘tighter’ cough than the wet cough that is usually seen in infective exacerbations of CF.

Diagnosis of ABPA requires specialist advice; it involves chest X-ray and serum-specific immunoglobulin E (IgE) tests (‘RAST’ tests). See International Society for Human and Animal Mycology (ISHAM) diagnostic criteria for allergic bronchopulmonary aspergillosis for diagnostic criteria.

If ABPA is suspected in a patient with CF, refer the patient to their CF physician for confirmation and early management.