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Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

Ibrahim Ahmed Janahi1,2, Abdul Rehman3, Amal Rashid Al-Naimi1,2

  • 1Department of Clinical Pediatrics, Weill Cornell Medical College-Qtar, Doha, Qatar.

Annals of Thoracic Medicine
|May 5, 2017
PubMed

Insights

Allergic bronchopulmonary aspergillosis (ABPA) affects cystic fibrosis (CF) patients, causing inflammation due to Aspergillus fumigatus. Early diagnosis and treatment with steroids, antifungals, or omalizumab are crucial for managing this underdiagnosed condition.

Area of Science:

  • Pulmonology
  • Allergy and Immunology
  • Infectious Diseases

Background:

  • Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disorder often seen in asthma and cystic fibrosis (CF) patients.
  • It involves an IgE-mediated response to Aspergillus fumigatus allergens, leading to airway inflammation, bronchospasm, and bronchiectasis in CF.
  • ABPA prevalence in CF is approximately 8.9%, but it's frequently underdiagnosed due to overlapping symptoms with CF.

Purpose of the Study:

  • To review the diagnosis and management of ABPA in patients with cystic fibrosis.
  • To highlight the challenges in diagnosing ABPA due to overlapping clinical features with CF.
  • To discuss current and potential therapeutic strategies for ABPA in CF.

Main Methods:

  • Literature review of studies on ABPA in CF patients.
  • Analysis of diagnostic criteria, including clinical, radiological, and serological markers.
  • Evaluation of treatment options: steroids, antifungals, and emerging therapies like omalizumab.

Main Results:

  • Diagnosis requires identifying clinical/radiological deterioration, elevated IgE, and Aspergillus sensitization.
  • Systemic steroids are the primary treatment for inflammation.
  • Antifungals are used for steroid-intolerant/refractory cases; omalizumab shows promise for reducing exacerbations.

Conclusions:

  • ABPA diagnosis in CF patients needs proactive consideration, especially with unexplained deterioration.
  • Management involves a stepwise approach with steroids, antifungals, and potentially omalizumab.
  • Further research, including randomized controlled trials, is essential to confirm omalizumab's efficacy in CF-ABPA.
Keywords:
Allergic bronchopulmonary aspergillosisallergic fungal mycosiscystic fibrosis

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