Highlights & Basics
- Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to bronchial colonization by Aspergillus fumigatus mold, typically affecting patients with an underlying predisposition, specifically asthma or cystic fibrosis.
- When predisposed individuals inhale the aspergillus spores, which are ubiquitous in the environment, the spores germinate in the lungs, setting off a complex immune cascade which results in the formation of immunoglobulin E and immunoglobulin G antibodies and immune-mediated lung destruction.
- ABPA usually presents in a similar way to asthma but may be complicated by bronchial obstruction, fever, malaise, expectoration of brownish mucus plugs, peripheral blood eosinophilia, and hemoptysis in later stages.
- Treatment involves corticosteroids with or without antifungal therapy. Monoclonal antibodies used for the treatment of asthma are under active investigation as a promising alternative treatment for ABPA.
- Early diagnosis and rapid initiation of treatment can prevent the development of bronchiectasis or pulmonary fibrosis that otherwise occurs in the later stages of the disease.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Chest x-ray in a patient with ABPA: ring shadows (long arrows) represent bronchiectatic airways seen in cross-section; tram lines (short arrow) seen longitudinally
Chest x-ray in a patient with ABPA: classic glove-finger pattern represents central bronchiectatic airways impacted with mucus
CT chest in a patient with ABPA: dilated, mucoid-impacted bronchiectatic airways
CT chest in a patient with ABPA: glove-finger shadow due to mucoid impaction in central bronchiectasis in a patient with asthma
Citations
Patterson TF, Thompson GR 3rd, Denning DW, et al. Practice Guidelines for the Diagnosis and Management of Aspergillosis: 2016 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2016 Aug 15;63(4):e1-e60.[Abstract][Full Text]
Pasteur MC, Bilton D, Hill AT; British Thoracic Society Bronchiectasis non-CF Guideline Group. British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010;65(suppl 1):i1-i58.[Abstract][Full Text]
Cystic Fibrosis Foundation. Allergic bronchopulmonary aspergillosis clinical care guidelines. 2003 [internet publication].[Full Text]
1. Agarwal R, Chakrabarti A, Shah A, et al. Allergic bronchopulmonary aspergillosis: review of literature and proposal of new diagnostic and classification criteria. Clin Exp Allergy. 2013 Aug;43(8):850-73.[Abstract][Full Text]
2. Mahdavinia M, Grammer LC. Management of allergic bronchopulmonary aspergillosis: a review and update. Ther Adv Respir Dis. 2012;6:173-187.[Abstract]
3. Agarwal R, Muthu V, Sehgal IS, et al. Prevalence of aspergillus sensitization and allergic bronchopulmonary aspergillosis in adults with bronchial asthma: a systematic review of global data. J Allergy Clin Immunol Pract. 2023 Jun;11(6):1734-51.e3.[Abstract]
4. Zander DS. Allergic bronchopulmonary aspergillosis: an overview. Arch Pathol Lab Med. 2005;129:924-928.[Abstract][Full Text]
5. Tillie-Leblond I, Tonnel AB. Allergic bronchopulmonary aspergillosis. Allergy. 2005;60:1004-1013.[Abstract][Full Text]
6. Kosmidis C, Denning DW. The clinical spectrum of pulmonary aspergillosis. Thorax. 2015 Mar;70(3):270-7.[Abstract][Full Text]
7. Shah A, Panjabi C. Allergic aspergillosis of the respiratory tract. Eur Respir Rev. 2014 Mar 1;23(131):8-29.[Abstract][Full Text]
8. Eppinger TM, Greenberger PA, White DA, et al. Sensitization to Aspergillus species in the congenital neutrophil disorders chronic granulomatous disease and hyper-IgE syndrome. J Allergy Clin Immunol. 1999;104:1265-1272.[Abstract]
9. Verleden GM, Dupont LJ, Van Raemdonck DE. ABPA: does it really exist after lung transplantation? J Heart Lung Transplant. 2009 Nov;28(11):1239.[Abstract]
10. Patterson K, Strek ME. Allergic bronchopulmonary aspergillosis. Proc Am Thorac Soc. 2010 May;7(3):237-44.[Abstract]
11. Denning DW, Pleuvry A, Cole DC. Global burden of allergic bronchopulmonary aspergillosis with asthma and its complication chronic pulmonary aspergillosis in adults. Med Mycol. 2013 May;51(4):361-70.[Abstract][Full Text]
12. Janahi IA, Rehman A, Al-Naimi AR. Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis. Ann Thorac Med. 2017 Apr-Jun;12(2):74-82.[Abstract][Full Text]
13. Cystic Fibrosis Foundation. Patient registry 1994 annual data report. Bethesda, MD: The Foundation; 1995.
14. Geller DE, Kaplowitz H, Light MJ, et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis: reported prevalence, regional distribution, and patient characteristics. Chest. 1999;116:639-646.[Abstract][Full Text]
15. Mastella G, Rainisio M, Harms HK, et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis. A European epidemiological study. Epidemiologic Registry of Cystic Fibrosis. Eur Respir J. 2000 Sep;16(3):464-71.[Abstract][Full Text]
16. Maturu VN, Agarwal R. Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: systematic review and meta-analysis. Clin Exp Allergy. 2015 Dec;45(12):1765-78.[Abstract][Full Text]
17. Greenberger PA, Bush RK, Demain JG, et al. Allergic bronchopulmonary aspergillosis. J Allergy Clin Immunol Pract. 2014;2:703-708.[Abstract]
18. Gago S, Denning DW, Bowyer P. Pathophysiological aspects of Aspergillus colonization in disease. Med Mycol. 2019 Apr 1;57(supplement_2):S219-27.[Abstract][Full Text]
19. Arias M, Santiago L, Vidal-García M, et al. Preparations for invasion: modulation of host lung immunity during pulmonary Aspergillosis by Gliotoxin and other fungal secondary metabolites. Front Immunol. 2018;9:2549.[Abstract][Full Text]
20. Knutsen AP, Slavin RG. Allergic bronchopulmonary aspergillosis in asthma and cystic fibrosis. Clin Dev Immunol. 2011;2011:843763.[Abstract][Full Text]
21. Virnig C, Bush RK. Allergic bronchopulmonary aspergillosis: a US perspective. Curr Opin Pulm Med. 2007;13:67-71.[Abstract]
22. Agarwal R, Sehgal IS, Dhooria S, et al. Developments in the diagnosis and treatment of allergic bronchopulmonary aspergillosis. Expert Rev Respir Med. 2016 Dec;10(12):1317-34.[Abstract]
23. Sunman B, Ademhan Tural D, Ozsezen B, et al. Current approach in the diagnosis and management of allergic bronchopulmonary aspergillosis in children with cystic fibrosis. Front Pediatr. 2020;8:582964.[Abstract][Full Text]
24. Jederlinic PJ, Sicilian L, Gaensler EA. Chronic eosinophilic pneumonia: a report of 19 cases and a review of the literature. Medicine (Baltimore). 1988;67:154-162.[Abstract]
25. Stevens DA, Moss RB, Kurup VP, et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis: state of the art: Cystic Fibrosis Foundation consensus conference. Clin Infect Dis. 2003;37(suppl 3):S225-S264.[Abstract][Full Text]
26. Patterson TF, Thompson GR 3rd, Denning DW, et al. Practice Guidelines for the Diagnosis and Management of Aspergillosis: 2016 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2016 Aug 15;63(4):e1-e60.[Abstract][Full Text]
27. Casaulta C, Fluckiger S, Crameri R, et al. Time course of antibody response to recombinant Aspergillus fumigatus antigens in cystic fibrosis with and without ABPA. Pediatr Allergy Immunol. 2005;16:217-225.[Abstract]
28. Agarwal R. Allergic bronchopulmonary aspergillosis. Chest. 2009;135:805-826.[Abstract]
29. Agarwal R, Saxena P, Muthu V, et al. Evaluation of simpler criteria for diagnosing allergic bronchopulmonary aspergillosis complicating asthma. Front Cell Infect Microbiol. 2022;12:861866.[Abstract][Full Text]
30. Pasteur MC, Bilton D, Hill AT; British Thoracic Society Bronchiectasis non-CF Guideline Group. British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010;65(suppl 1):i1-i58.[Abstract][Full Text]
31. Agarwal R, Gupta D, Aggarwal AN, et al. Clinical significance of hyperattenuating mucoid impaction in allergic bronchopulmonary aspergillosis: an analysis of 155 patients. Chest. 2007 Oct;132(4):1183-90.[Abstract]
32. Agarwal R, Maskey D, Aggarwal AN, et al. Diagnostic performance of various tests and criteria employed in allergic bronchopulmonary aspergillosis: a latent class analysis. PLoS One. 2013;8(4):e61105.[Abstract][Full Text]
33. Rosenberg M, Patterson R, Mintzer R, et al. Clinical and immunologic criteria for the diagnosis of allergic bronchopulmonary aspergillosis. Ann Intern Med. 1977 Apr;86(4):405-14.[Abstract]
34. Cystic Fibrosis Foundation. Allergic bronchopulmonary aspergillosis clinical care guidelines. 2003 [internet publication].[Full Text]
35. Asano K, Hebisawa A, Ishiguro T, et al. New clinical diagnostic criteria for allergic bronchopulmonary aspergillosis/mycosis and its validation. J Allergy Clin Immunol. 2021 Apr;147(4):1261-8.e5.[Abstract][Full Text]
36. Marchand E, Reynaud-Gaubert M, Lauque D, et al. Idiopathic chronic eosinophilic pneumonia: a clinical and follow-up study of 62 cases. The Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Medicine (Baltimore). 1998;77:299-312.[Abstract]
37. Cottin V, Frognier R, Monnot H, et al. Chronic eosinophilic pneumonia after radiation therapy for breast cancer. Eur Respir J. 2004;23:9-13.[Abstract][Full Text]
38. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999;78:26-37.[Abstract]
39. Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet. 2003;361:587-594.[Abstract]
40. Keogh KA, Specks U. Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23:3-12.[Abstract]
41. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994;83:2759-2779.[Abstract][Full Text]
42. Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003;348:1201-1214.[Abstract][Full Text]
43. Agarwal R, Khan A, Gupta D, et al. An alternate method of classifying allergic bronchopulmonary aspergillosis based on high-attenuation mucus. PLoS One. 2010 Dec 15;5(12):e15346.[Abstract][Full Text]
44. Patterson R, Greenberger PA, Radin RC, et al. Allergic bronchopulmonary aspergillosis: staging as an aid to management. Ann Intern Med. 1982;96:286-291.[Abstract]
45. Agarwal R, Gupta D, Aggarwal AN, et al. Allergic bronchopulmonary aspergillosis: lessons from 126 patients attending a chest clinic in north India. Chest. 2006;130:442-448.[Abstract][Full Text]
46. Safirstein BH, D'Souza MF, Simon G, et al. Five-year follow-up of allergic bronchopulmonary aspergillosis. Am Rev Respir Dis. 1973;108:450-459.[Abstract]
47. Greenberger PA. Allergic bronchopulmonary aspergillosis. J Allergy Clin Immunol. 2002;110:685-692.[Abstract][Full Text]
48. Ricketti AJ, Greenberger PA, Patterson R. Serum IgE as an important aid in management of allergic bronchopulmonary aspergillosis. J Allergy Clin Immunol. 1984;74:68-71.[Abstract]
49. Agarwal R, Dhooria S, Singh Sehgal I, et al. A randomized trial of itraconazole vs prednisolone in acute-stage allergic bronchopulmonary aspergillosis complicating asthma. Chest. 2018 Mar;153(3):656-64.[Abstract]
50. Chishimba L, Niven RM, Cooley J, et al. Voriconazole and posaconazole improve asthma severity in allergic bronchopulmonary aspergillosis and severe asthma with fungal sensitization. J Asthma. 2012 May;49(4):423-33.[Abstract][Full Text]
51. Skov M, Main KM, Sillesen IB, et al. Iatrogenic adrenal insufficiency as a side-effect of combined treatment of itraconazole and budesonide. Eur Respir J. 2002;20:127-133.[Abstract][Full Text]
52. Blanco-Dorado S, Marques Afonso AT, Bandín-Vilar EJ, et al. Voriconazole hepatotoxicity as a result of steroid withdrawal in a patient with allergic bronchopulmonary aspergillosis. Br J Clin Pharmacol. 2019 Feb;85(2):460-462.[Abstract][Full Text]
53. Muthu V, Dhooria S, Sehgal IS, et al. Nebulized amphotericin B for preventing exacerbations in allergic bronchopulmonary aspergillosis: a systematic review and meta-analysis. Pulm Pharmacol Ther. 2023 Aug;81:102226.[Abstract][Full Text]
54. British Thoracic Association. Inhaled beclomethasone dipropionate in allergic bronchopulmonary aspergillosis. Report to the Research Committee of the British Thoracic Association. Br J Dis Chest. 1979;73:349-356.[Abstract]
55. Seaton A, Seaton RA, Wightman AJ. Management of allergic bronchpulmonary aspergillosis without maintenance oral corticosteroids: a fifteen-year follow-up. QJM. 1994;87:529-537.[Abstract]
56. Darragh K, Akuthota P. Corticosteroid-sparing effect of biologics in patients with allergic bronchopulmonary aspergillosis. Ann Allergy Asthma Immunol. 2024 Jan 15:S1081-1206(24)00012-7.[Abstract][Full Text]
57. Kyriakidis I, Tragiannidis A, Munchen S, et al. Clinical hepatotoxicity associated with antifungal agents. Expert Opin Drug Saf. 2017 Feb;16(2):149-65.[Abstract][Full Text]
58. Tverdek FP, Kofteridis D, Kontoyiannis DP. Antifungal agents and liver toxicity: a complex interaction. Expert Rev Anti Infect Ther. 2016 Aug;14(8):765-76.[Abstract]
59. Zirbes JM, Milla CE. Steroid-sparing effect of omalizumab for allergic bronchopulmonary aspergillosis and cystic fibrosis. Pediatr Pulmonol. 2008;43:607-610.[Abstract]
60. Kanu A, Patel K. Treatment of allergic bronchopulmonary aspergillosis (ABPA) in CF with anti-IgE antibody (omalizumab). Pediatr Pulmonol. 2008;43:1249-1251.[Abstract]
61. van der Ent CK, Hoekstra H, Rijkers GT. Successful treatment of allergic bronchopulmonary aspergillosis with recombinant anti-IgE antibody. Thorax. 2007;62:276-277.[Abstract]
62. Moss RB. The use of biological agents for the treatment of fungal asthma and allergic bronchopulmonary aspergillosis. Ann N Y Acad Sci. 2012;1272:49-57.[Abstract][Full Text]
63. Lötvall J, Akdis CA, Bacharier LB, et al. Asthma endotypes: a new approach to classification of disease entities within the asthma syndrome. J Allergy Clin Immunol. 2011;127:355-360.[Abstract][Full Text]
64. Jat KR, Walia DK, Khairwa A. Anti-IgE therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2021 Sep 22;9(9):CD010288.[Abstract]
65. Schleich F, Vaia ES, Pilette C, et al. Mepolizumab for allergic bronchopulmonary aspergillosis: report of 20 cases from the Belgian Severe Asthma Registry and review of the literature. J Allergy Clin Immunol Pract. 2020 Jul-Aug;8(7):2412-3.e2.[Abstract]
66. Dhariwal J, Hearn AP, Kavanagh JE, et al. Real-world effectiveness of anti-IL-5/5R therapy in severe atopic eosinophilic asthma with fungal sensitization. J Allergy Clin Immunol Pract. 2021 Jun;9(6):2315-20.e1.[Abstract]
67. Tolebeyan A, Mohammadi O, Vaezi Z, et al. Mepolizumab as possible treatment for allergic bronchopulmonary aspergillosis: a review of eight cases. Cureus. 2020 Aug 12;12(8):e9684.[Abstract][Full Text]
68. Mikura S, Saraya T, Yoshida Y, et al. Successful treatment of mepolizumab- and prednisolone-resistant allergic bronchopulmonary aspergillosis with dupilumab. Intern Med. 2021 Sep 1;60(17):2839-42.[Abstract][Full Text]
69. ClinicalTrials.gov. Investigating treatment with dupilumab in patients with allergic bronchopulmonary aspergillosis (ABPA) (LIBERTY ABPA AIRED). Aug 2024 [internet publication].[Full Text]
70. Soeda S, Kono Y, Tsuzuki R, et al. Allergic bronchopulmonary aspergillosis successfully treated with benralizumab. J Allergy Clin Immunol Pract. 2019 May-Jun;7(5):1633-5.[Abstract]
71. Tomomatsu K, Sugino Y, Okada N, et al. Rapid clearance of mepolizumab-resistant bronchial mucus plugs in allergic bronchopulmonary aspergillosis with benralizumab treatment. Allergol Int. 2020 Oct;69(4):636-8.[Abstract][Full Text]
72. Cockrill BA, Hales CA. Allergic bronchopulmonary aspergillosis. Annu Rev Med. 1999;50:303-316.[Abstract]
73. American Lung Association Asthma Clinical Research Centers, Mastronarde JG, Anthonisen NR, et al. Efficacy of esomeprazole for treatment of poorly controlled asthma. N Engl J Med. 2009;360:1487-1499.[Abstract]
74. National Asthma Education and Prevention Program. Expert Panel report 3 (EPR-3): guidelines for the diagnosis and management of asthma: summary report 2007. J Allergy Clin Immunol. 2007;120(5 suppl):S94-S138.[Abstract]
75. Basich JE, Graves TS, Baz MN, et al. Allergic bronchopulmonary aspergillosis in corticosteroid-dependent asthmatics. J Allergy Clin Immunol. 1981;68:98-102.[Abstract]
76. Agarwal R, Aggarwal AN, Dhooria S. A randomised trial of glucocorticoids in acute-stage allergic bronchopulmonary aspergillosis complicating asthma. Eur Respir J. 2016;47:490-498.[Abstract]
77. Moss RB. Pathophisiology and immunology of allergic bronchopulmonary aspergillosis. Med Mycol. 2005;43(suppl 1):S203-S206.[Abstract]
78. Liu D, Ahmet A, Ward L, et al. A practical guide to the monitoring and management of the complications of systemic corticosteroid therapy. Allergy Asthma Clin Immunol. 2013 Aug 15;9(1):30.[Abstract][Full Text]
79. Leon EE, Craig TJ. Antifungals in the treatment of allergic bronchopulmonary aspergillosis. Ann Allergy Asthma Immunol. 1999;82:511-516.[Abstract]
80. Gilley SK, Goldblatt MR, Judson MA. The treatment of ABPA. In: Pasqualotto AC, ed. Aspergillosis: from diagnosis to prevention. London, New York: Springer; 2009:747-59.
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