Updated: 3/30/2019

Allergic Bronchopulmonary Aspergillosis (ABPA)

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Snapshot
  • A 14-year-old boy with past medical history of cystic fibrosis presents to his pulmonologist for a new cough. He reports that for the past few weeks, he has been coughing up brown sputum more and experiencing low-grade fevers. A recent chest radiograph showed a left lower lobe consolidation, and he completed a course of antibiotics without improvement. He also recently underwent testing for tuberculosis, which was all negative. On physical exam, there is wheezing and crackles bilaterally. A chest computed tomography (CT) shows central bronchiectasis and a skin test for Aspergillus causes a wheal 5 mm in diameter to form on his skin.
Introduction
  • Clinical definition
    • hypersensitivity syndrome due to Aspergillus fumigatus, characterized by asthma, eosinophilia, recurrent pulmonary infiltrates, and bronchiectasis
  • Epidemiology
    • demographics
      • occurs in patients with pre-existing pulmonary disease
      • more common in patients with cystic fibrosis
    • risk factors
      • cystic fibrosis
      • asthma
  • Pathogenesis
    • mechanism
      • predominantly a type I hypersensitivity reaction
  • Associated conditions
    • atopic diseases
      • atopic dermatitis
      • allergic rhinitis
      • asthma
      • food allergies
    • hyper IgE syndrome
 Presentation
  • Symptoms
    • systemic symptoms
      • fatigue
      • low-grade fevers
    • chronic asthma refractory to medical therapy
    • new or worsening cough
    • brown/black sputum
    • hemoptysis
  • Physical exam
    • dyspnea
    • wheezing
    • crackles
 Studies
  • Diagnostic testing
    • diagnostic approach
      • Aspergillus skin test is used to screen for hypersensitivity, but further imaging and lab tests are recommended
    • imaging 
      • chest radiograph
        • often performed as the first imaging
        • transient consolidations (fleeting shadows)
        • finger-in-glove sign showing dilated bronchioles
      • chest computed tomography (CT)
        • gold standard
        • central bronchiectasis with normal peripheral bronchi
        • finger-in-glove sign showing mucoid impaction in bronchioles
    • studies
      • positive Aspergillus skin test (wheal)
      • ↑ eosinophil and IgE levels
      • Aspergillus-specific antibodies IgG and IgE
      • precipitin antibodies
  • Diagnostic criteria
    • multiple diagnostic criteria exist
      • e.g., Rosenberg-Patterson diagnostic criteria for patients without cystic fibrosis: at least 6 must be met
        • asthma, transient pulmonary opacities on imaging, positive Aspergillus skin testing, peripheral eosinophilia, positive Aspergillus-specific antibodies IgG or IgE, elevated peripheral IgE, bronchiectasis on imaging, or positive precipitin antibodies
    • based on clinical presentation and imaging
      • requires new findings on imaging that do not clear with antibiotics in patients with cystic fibrosis
 Differential
  • Tuberculosis
    • distinguishing factor
      • positive tuberculin skin test
      • no peripheral eosinophilia
Treatment
  • Management approach
    • aimed at symptoms control and prevention of exacerbation
  • First-line
    • systemic corticosteroids
  • Second-line
    • itraconazole
      • indication
        • severe disease refractory to solo steroid therapy
  • Other treatments
    • omalizumab
      • may reduce exacerbations
Complications
  • Respiratory failure
  • Pulmonary hypertension
  • Pneumothorax

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