II. Epidemiology
- Worldwide Prevalence: 4 Million
III. Pathophysiology
- Immunologic disorder caused by Hypersensitivity to Aspergillus fumigatus
- Aspergillus fumigatus colonization of airways and mucus resulting in Asthma and Bronchiectasis
- A. fumigatus germinate into hyphae, resulting in T Cell response
- A. fumigatus is most common, but A. niger, A. flavus and A. clavatus may also cause ABPA
-
Bronchial Asthma
- Due to IgE mediated Hypersensitivity
-
Bronchiectasis
- Due to immune complex deposition in proximal airways
IV. Risk Factors
- Cystic Fibrosis
- Asthma
- Immunocompromised State
- Genetic predisposition (esp. HLA-DR2 or HLA-DR5)
V. Symptoms
VI. Signs
- Wheezing
- Rhonchi
- Rales (Bronchiectasis, esp. Cystic Fibrosis)
VII. Associated Conditions
VIII. Labs
- Peripheral Eosinophilia
- Serum precipitans to a. fumigatus
- Serum Aspergillus >27 mg/L
- Serum IgE increased
- Total Serum IgE typically >1000 ng/ml
-
Aspergillus skin test
- Immediate wheal and flare reaction to a. fumigatus
IX. Imaging
-
Chest XRay
- Recurrent and fleeting Pulmonary Infiltrates and Bronchiectasis primarily in the upper lobes
- High Resolution CT Chest
- Preferred to assist in diagnosis, exclude other causes, identify Bronchiectasis distribution
X. Diagnosis
- Multiple diagnostic protocols based on clinical presentation, lab and imaging
- Bronchial Asthma
- Central Bronchiectasis
- Fleeting Pulmonary Infiltrates
- ISHAM Criteria
- Bronchial Asthma or Cystic Fibrosis AND
- Type 1 Aspergillus skin test reaction or elevated serum IgE to A. fumigatus AND
- Two of three minor criteria positive
- Serum IgG or IgM Antibody to A. fumigatus
- Imaging opacities consistent with ABPA
- Total serum Eosinophils >500 IU/ml if steroid naive (otherwise >1000 IU/ml)
- Rosenberg-Patterson Criteria (1977)
- Diagnosis: 6 of 8 major criteria positive is highly suggestive of Allergic Bronchopulmonary Aspergillosis
- Major Criteria (Mnemonic: ARTEPICS)
- Asthma (Bronchial)
- Radiographs with fleeting pulmonary opacities
- Test positive for immediate cutaneous Hypersensitivity to AspergillusAntigen (wheal and flare reaction)
- Eosinophilia in peripheral blood (>1000/mm3)
- Precipitating Antibody to AspergillusAntigen
- IgE serum levels increased (>1000 IU/ml)
- Central or proximal Bronchiectasis (medial two thirds of a high resolution CT)
- Serum A Fumigatus specific IgG and IgE levels >2 S.D. above that of Aspergillus hypersensitive Asthma samples
- Minor Criteria
- Aspergillus delayed skin reaction (type 3)
- Brownish-black mucus plugs
- Sputum positive for A Fumigatus
- Sputum Culture positive or fungal hyphae and Eosinophils on microscopy
- References
- Boloor and Nayak (2018) Exam Preparatory Manual, Jaypee Brothers Medical Pub
- Patel (2019) Cureus 11(4):e4550 +PMID: 31275774 [PubMed]
- Saxena (2021) J Allergy Clin Immunol Pract 9(1):328-35 +PMID: 32890756 [PubMed]
XI. Differential Diagnosis
- Severe Asthma
- COPD
- Cystic Fibrosis
- Bronchiectasis
- Churg-Strauss Syndrome
- Eosinophilic Pneumonia
- Pulmonary Tuberculosis
- Hypersensitivity Pneumonitis
- Other Fungal Pneumonia
XII. Management
- Early diagnosis and management offers best prognosis (before Bronchiectasis develops)
- Long-term use of Systemic Corticosteroids
- Starting at 0.5 to 1 mg/kg for first 2 weeks
- Slow taper over 3-5 months
- Oral Antifungals
- Itraconazole 200 mg twice daily for 16 weeks
- Often continued at 200 mg daily or every other day while Corticosteroid dependent
- Other Antifungals
- Itraconazole 200 mg twice daily for 16 weeks
- Other medications to consider
- Omalizumab (esp. in Asthma)
XIII. Compolications
- Severe, refractory Asthma
- Invasive Aspergillosis or chronic pulmonary Aspergillosis
- Pulmonary Fibrosis
- Emphysema
- Recurrent lung infection
XIV. References
- Sisodia and Bajaj (2021) Allergic Bronchopulmonary Aspergillosis, StatPearls, accessed 11/12/2021
- Stevens (2003) Clin Infect Dis 37:S225-64 [PubMed]
- Agarwal (2013) Clin Exp Allergy 43(8):850-73 [PubMed]
- Agarwal (2016) Expert Rev Respir Med 10(12):1317-34 [PubMed]
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Related Studies
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 |
Russian | ALLERGICHESKII BRONKHOLEGOCHNYI ASPERGILLEZ, ASPERGILLEZ ALLERGICHESKII BRONKHOLEGOCHNYI, АЛЛЕРГИЧЕСКИЙ БРОНХОЛЕГОЧНЫЙ АСПЕРГИЛЛЕЗ, АСПЕРГИЛЛЕЗ АЛЛЕРГИЧЕСКИЙ БРОНХОЛЕГОЧНЫЙ |
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 |