Disease name and synonyms 

Allergic bronchopulmonary aspergillosis (ABPA)

Fungi responsible (links to these)

Aspergillus fumigatus (>95%), A. terreus, A. niger and A. flavus

Disease description

Worsening asthma, worsening cystic fibrosis, coughing paroxysms relieved by coughing up plugs or very thick mucous. Occasionally presents as mucoid impaction, with consolidation of the lung. Recurrent ‘chest infections’ which are in reality obstructed lung segments occasionally occur. May be found on screening for IgE, or incidentally when complications occur. 

Frequency and global burden

Worldwide. Uncommon in childhood, but described. Frequency estimates vary from 0.7 to 3.5% of consecutive asthma patients referred to a chest specialist. 12-15% of adolescent and adult CF patients. An estimate of 4,800,000 adults with ABPA compliating asthma has been made.

Underlying problems and at risk patients

Asthma and cystic fibrosis. Rarely no underlying disease.

Diagnostic testing

IgE >1,000 KIU/L plus either a skin test of specific IgE test for fungi and continuing respiratory symptoms. May have eosinophilia and/or Aspergillus IgG antibodies. Sputum cultures positive for Aspergillus in 20-60%, on multiple specimens. Aspergillus PCR usually positive in sputum or bronchoscopy specimens.

Treatments

Oral corticosteroids for exacerbation. Inhaled steroids for maintenance. Oral itraconazole (or another mould active azole) for improved health status and reduced corticosteroid usage. Hypertonic saline (6 or7%) nebuliser for clearing thick sputum. Azithromycin for continuing irritable cough.

Outlook and prognosis

Excellent prognosis and few symptoms if well controlled. Complication of bronchiectasis in asthma common and may be present at diagnosis. Usually centrally located and somewhat distinctive on CT scan. Chronic pulmonary aspergillosis probably occurs in ~10% of ABPA patients, but is slow in onset and can be atypical with localized pleural fibrosis and lung shrinkage predominating over cavitation initially.