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Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis

S Mroueh1, A Spock

  • 1Department of Pediatrics, Duke University Medical Center, Durham, NC 27710.

Chest
|January 1, 1994
PubMed

Insights

Allergic bronchopulmonary aspergillosis (ABPA) affects 6.5% of cystic fibrosis (CF) patients, often presenting with severe disease. Steroid therapy typically improves symptoms and lung function in these patients.

Area of Science:

  • Pulmonology
  • Infectious Diseases
  • Immunology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
  • Allergic Bronchopulmonary Aspergillosis (ABPA) is a hypersensitivity reaction to Aspergillus fumigatus, often seen in CF patients.
  • Accurate incidence and diagnostic criteria for ABPA in CF are crucial for effective management.

Purpose of the Study:

  • To determine the incidence of ABPA in patients with CF.
  • To identify clinical characteristics and diagnostic features of ABPA in this population.
  • To evaluate the response to steroid therapy in CF patients with ABPA.

Main Methods:

  • Retrospective review of 236 patients with CF at Duke CF Center.
  • Identification of patients with Aspergillus fumigatus colonies.
  • Analysis of clinical data, diagnostic markers, and treatment outcomes for ABPA diagnosis.

Main Results:

  • Sixty patients (25%) had Aspergillus fumigatus; these patients were older with more severe CF (lower Shwachman-Kulczycki scores).
  • Fifteen patients (6.5% of total) were diagnosed with ABPA, showing similar age and S-K scores to those with A. fumigatus but no ABPA.
  • Diagnostic features included refractory wheezing, infiltrates, eosinophilia, positive skin tests, and elevated IgE. Steroid therapy led to clinical improvement within 1 month, though IgE levels did not consistently decrease.

Conclusions:

  • ABPA is a significant complication in CF, occurring in 6.5% of patients in this cohort.
  • While diagnosis can be challenging, key clinical and laboratory findings aid identification.
  • Most CF patients with ABPA respond well to steroid therapy, with notable improvements in symptoms and lung function.

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