This entity is seen is almost only encountered in patients with longstanding asthma, and only occasionally in patients with cystic fibrosis 4-5. Only rarely does it appear in patients with no other identifiable pulmonary illness 5.
In general, patients are young and are diagnosed before the age of 40 years 9.
Clinically, patients have atopic symptoms (especially asthma) and present with recurrent chest infection. They may expectorate orange-coloured mucous plugs.
A clinical staging system has been developed 9:
- stage I: acute
- stage II: remission
- stage III: recurrent exacerbation
- stage IV: steroid-dependent asthma
- stage V: pulmonary fibrosis
Major and minor criteria have also been established 5-6.
- major criteria
- minor criteria
- fungal elements in sputum
- expectoration of brown plugs/flecks
- delayed skin reactivity to fungal antigens
Allergic bronchopulmonary aspergillosis (ABPA) is the result of hypersensitivity towards Aspergillus spp which grows within the lumen of the bronchi, without invasion. The hypersensitivity initially causes bronchospasm and bronchial wall oedema, which is IgE-mediated. Ultimately, there is bronchial wall damage with loss of muscle and bronchial wall cartilage resulting in bronchiectasis (typically central bronchiectasis) 7. Both type I and III allergic reactions have been implicated 4.
Bronchocentric granulomatosis often occurs, which is characterised by necrotizing granulomatous inflammation that destroys the walls of small bronchi and bronchioles. Macroscopically, the mucous plugs are orange / brown in colour.
Segmental and subsegmental bronchi are dilated and filled with mucous, admixed with eosinophils and occasional fungal hyphae 4,7. Charcot-Leyden crystals may be prominent 7.
Laboratory findings include:
- elevated Aspergillus-specific IgE
- elevated precipitating IgG against Aspergillus
- peripheral eosinophilia
- positive skin test
Early in the disease chest x-rays will appear normal, or only demonstrate changes of asthma. Transient patchy areas of consolidation may be evident representing eosinophilic pneumonia.
Eventually, bronchiectasis may be evident. Mucoid impaction in dilated bronchi can appear mass-like or sausage shaped or branching opacities (finger in glove sign). Pulmonary collapse may be seen as a consequence of endobronchial mucoid impaction.
CT findings include:
- fleeting pulmonary alveolar opacities: common
- centrilobular nodules representing dilated and opacified bronchioles 4
- central, upper lobe saccular bronchiectasis involving segmental and subsegmental bronchi is characteristic
- mucoid impaction results in a bronchocoele, the finger in glove sign
- this may give a Y, V or toothpaste-like like configuration
- centrilobular nodular opacities.
- high attenuation (calcification) in impacted mucus in ~30% 3-4
- bronchial wall thickening: common
- chronic disease may progress to pulmonary fibrosis, predominantly in the upper lobe
- cavitation: 10%
Treatment and prognosis
Treatment of ABPA is difficult due to the ubiquity of Aspergillus in the environment. The main focus of treatment revolves around 8:
- managing asthma
- limiting/controlling exacerbations: corticosteroid plays a major role
- eradicating Aspergillus from the airway: antifungals, e.g. Ketoconazole
- preventing late complications, e.g. severe bronchiectasis, fibrosis
Many patients are successfully managed after diagnosis and never progress to stage IV or V. In stages I to III prognosis is excellent, whereas stage V has a high 5-year mortality from respiratory failure 9.
For mucoid impaction consider:
- 1. Weissleder R, Wittenberg J, Harisinghani MG et-al. Primer of diagnostic imaging. Mosby Inc. (2007) ISBN:0323040683. Read it at Google Books - Find it at Amazon
- 2. Jeong YJ, Kim KI, Seo IJ et-al. Eosinophilic lung diseases: a clinical, radiologic, and pathologic overview. Radiographics. 27 (3): 617-37. doi:10.1148/rg.273065051 - Pubmed citation
- 3. Franquet T, Müller NL, Giménez A et-al. Spectrum of pulmonary aspergillosis: histologic, clinical, and radiologic findings. Radiographics. 21 (4): 825-37. Radiographics (full text) - Pubmed citation
- 4. Müller NL, Franquet T, Lee KS et-al. Imaging of pulmonary infections. Lippincott Williams & Wilkins. (2007) ISBN:078177232X. Read it at Google Books - Find it at Amazon
- 5. Collins J, Stern EJ. Chest radiology, the essentials. Lippincott Williams & Wilkins. (2007) ISBN:0781763142. Read it at Google Books - Find it at Amazon
- 6. Rosenberg M, Patterson R, Mintzer R et-al. Clinical and immunologic criteria for the diagnosis of allergic bronchopulmonary aspergillosis. Ann. Intern. Med. 1977;86 (4): 405-14. Pubmed citation
- 7. Zander DS. Allergic bronchopulmonary aspergillosis: an overview. Arch. Pathol. Lab. Med. 2005;129 (7): 924-8. Pubmed citation
- 8. Pasqualotto AC. Aspergillosis, From Diagnosis to Prevention. Springer Verlag. (2009) ISBN:9048124077. Read it at Google Books - Find it at Amazon
- 9. Grammer LC, Greenberger PA. Patterson's Allergic Diseases. Lippincott Williams & Wilkins. (2009) ISBN:0781794250. Read it at Google Books - Find it at Amazon
- 10. Agarwal R, Khan A, Garg M et-al. Chest radiographic and computed tomographic manifestations in allergic bronchopulmonary aspergillosis. World J Radiol. 2012;4 (4): 141-50. doi:10.4329/wjr.v4.i4.141 - Free text at pubmed - Pubmed citation
- 11. Silva CI, Colby TV, MüLler NL. Asthma and associated conditions: high-resolution CT and pathologic findings. AJR Am J Roentgenol. 2004;183 (3): 817-24. doi:10.2214/ajr.183.3.1830817 - Pubmed citation
- CNS aspergillosis
- fungal sinusitis
- allergic bronchopulmonary aspergillosis (ABPA)
- chronic necrotising pulmonary aspergillosis (CNPA) (or subacute invasive pulmonary aspergillosis or semi-invasive aspergillosis)
- airway invasive aspergillosis (or bronchopneumonic aspergillosis)
- angioinvasive aspergillosis
chronic pulmonary aspergillosis (CPA) - non-immunocompromised patients
- chronic cavitary pulmonary aspergillosis (CCPA)
- chronic necrotising pulmonary aspergillosis (CNPA)
- chronic fibrosing pulmonary aspergillosis (CFPA): may progress to this from CCPA in untreated
- obstructive bronchopulmonary aspergillosis