II. Epidemiology

  1. Worldwide Prevalence: 4 Million

III. Pathophysiology

  1. Immunologic disorder caused by Hypersensitivity to Aspergillus fumigatus
    1. Aspergillus fumigatus colonization of airways and mucus resulting in Asthma and Bronchiectasis
    2. A. fumigatus germinate into hyphae, resulting in T Cell response
    3. A. fumigatus is most common, but A. niger, A. flavus and A. clavatus may also cause ABPA
  2. Bronchial Asthma
    1. Due to IgE mediated Hypersensitivity
  3. Bronchiectasis
    1. Due to immune complex deposition in proximal airways

IV. Risk Factors

  1. Cystic Fibrosis
  2. Asthma
  3. Immunocompromised State
  4. Genetic predisposition (esp. HLA-DR2 or HLA-DR5)

V. Symptoms

  1. General
    1. Anorexia
    2. Fatigue
    3. Generalized myalgias
    4. Low grade fever
    5. Weight loss
  2. Respiratory
    1. Wheezing (related to Asthma)
    2. Productive Cough
      1. Brown mucus plugs
      2. Blood-tinged Sputum
    3. Dyspnea
    4. Pleuritic Chest Pain

VI. Signs

  1. Wheezing
  2. Rhonchi
  3. Rales (Bronchiectasis, esp. Cystic Fibrosis)

VII. Associated Conditions

VIII. Labs

  1. Peripheral Eosinophilia
  2. Serum precipitans to a. fumigatus
    1. Serum Aspergillus >27 mg/L
  3. Serum IgE increased
    1. Total Serum IgE typically >1000 ng/ml
  4. Aspergillus skin test
    1. Immediate wheal and flare reaction to a. fumigatus

IX. Imaging

  1. Chest XRay
    1. Recurrent and fleeting Pulmonary Infiltrates and Bronchiectasis primarily in the upper lobes
  2. High Resolution CT Chest
    1. Preferred to assist in diagnosis, exclude other causes, identify Bronchiectasis distribution

X. Diagnosis

  1. Multiple diagnostic protocols based on clinical presentation, lab and imaging
    1. Bronchial Asthma
    2. Central Bronchiectasis
    3. Fleeting Pulmonary Infiltrates
  2. ISHAM Criteria
    1. Bronchial Asthma or Cystic Fibrosis AND
    2. Type 1 Aspergillus skin test reaction or elevated serum IgE to A. fumigatus AND
    3. Two of three minor criteria positive
      1. Serum IgG or IgM Antibody to A. fumigatus
      2. Imaging opacities consistent with ABPA
      3. Total serum Eosinophils >500 IU/ml if steroid naive (otherwise >1000 IU/ml)

XII. Management

  1. Early diagnosis and management offers best prognosis (before Bronchiectasis develops)
  2. Long-term use of Systemic Corticosteroids
    1. Starting at 0.5 to 1 mg/kg for first 2 weeks
    2. Slow taper over 3-5 months
  3. Oral Antifungals
    1. Itraconazole 200 mg twice daily for 16 weeks
      1. Often continued at 200 mg daily or every other day while Corticosteroid dependent
    2. Other Antifungals
      1. Voriconazole
      2. Posaconazole
  4. Other medications to consider
    1. Omalizumab (esp. in Asthma)

XIII. Compolications

  1. Severe, refractory Asthma
  2. Invasive Aspergillosis or chronic pulmonary Aspergillosis
  3. Pulmonary Fibrosis
  4. Emphysema
  5. Recurrent lung infection

XIV. References

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Related Studies

Ontology: Aspergillosis, Allergic Bronchopulmonary (C0004031)

Definition (MSH) Hypersensitivity reaction (ALLERGIC REACTION) to fungus ASPERGILLUS in an individual with long-standing BRONCHIAL ASTHMA. It is characterized by pulmonary infiltrates, EOSINOPHILIA, elevated serum IMMUNOGLOBULIN E, and skin reactivity to Aspergillus antigen.
Definition (NCI) An immune reaction to aspergillus in patients usually suffering from asthma or cystic fibrosis.
Concepts Disease or Syndrome (T047)
MSH D001229
ICD9 518.6
ICD10 B44.81
SnomedCT 37981002
English Allergic Aspergilloses, Bronchopulmonary, Allergic Aspergillosis, Bronchopulmonary, Allergic Bronchopulmonary Aspergilloses, Allergic Bronchopulmonary Aspergillosis, Aspergilloses, Allergic Bronchopulmonary, Aspergilloses, Bronchopulmonary Allergic, Aspergillosis, Bronchopulmonary Allergic, Bronchopulmonary Allergic Aspergilloses, Bronchopulmonary Allergic Aspergillosis, Bronchopulmonary Aspergilloses, Allergic, Bronchopulmonary Aspergillosis, Allergic, ABPA - Allerg br-pul aspergill, Allerg bron-pul aspergillosis, Aspergillosis, Allergic Bronchopulmonary, ASPERG ALLERGIC BRONCHOPULM, ALLERGIC BRONCHOPULM ASPERG, BRONCHOPULM ASPERG ALLERGIC, ASPERG BRONCHOPULM ALLERGIC, allergic bronchopulmonary aspergillosis (diagnosis), allergic bronchopulmonary aspergillosis, ABPA, Bronchopulmonary aspergillosis allergic, Alrgc brncpul asprglosis, Aspergillosis, Allergic Bronchopulmonary [Disease/Finding], allergic bronchopulmonary aspergillosis (ABPA), abpa, allergic lung reaction to the fungus aspergillus, Allergic bronchopulmonary aspergillosis, ABPA - Allergic bronchopulmonary aspergillosis, Allergic bronchopulmonary aspergillosis (disorder)
Italian Aspergillosi broncopolmonare allergica, Aspergillosi allergica broncopolmonare
Dutch allergische bronchopulmonaire aspergillose, bronchopulmonaire aspergillosisallergie, Allergische bronchopulmonale aspergillose, Aspergillose, allergische bronchopulmonale, Bronchopulmonale aspergillose, Bronchopulmonale aspergillose, allergische
French Aspergillose broncho-pulmonaire allergique (ABPA), ABPA, Aspergillose bronchopulmonaire allergique, Aspergillose broncho-pulmonaire allergique
German ABPA, allergische bronchopulmonale Aspergillose, Allergische bronchopulmonale Aspergillose, Aspergillose, allergische bronchopulmonale, Bronchopulmonale Aspergillose
Spanish ABPA, aspergilosis broncopulmonar alérgica (trastorno), aspergilosis broncopulmonar alérgica, Aspergilosis broncopulmonar alérgica, Aspergilosis Broncopulmonar Alérgica
Swedish Aspergillos, allergisk bronkopulmonell
Japanese アレルギーセイキカンシハイアスペルギルスショウ, アスペルギルス症-アレルギー性気管支肺, アレルギー性気管支・肺アスペルギルス症, アレルギー性気管支肺アスペルギルス症, 気管支肺アスペルギルス症-アレルギー性, 気管支肺アスペルギルス症, アスペルギルス症-気管支肺
Czech aspergilóza alergická bronchopulmonální, Alergická bronchopulmonální aspergilóza, ABPA
Finnish Allerginen bronkopulmonaalinen aspergilloosi
Polish Aspergiloza alergiczna oskrzelowo-płucna
Hungarian ABPA, allergiás bronchopulmonalis aspergillosis, Allergiás bronchopulmonaris aspergillosis
Norwegian Allergisk bronkopulmonal aspergillose, Aspergillose, allergisk bronkopulmonal
Portuguese Aspergilose broncopulmonar alérgica, Aspergilose Broncopulmonar Alérgica