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allergic bronchopulmonary aspergillosis; allergic bronchopulmonary mycosis (ABPA)

Etiology: 1) risk factors - frequently develops in patients with asthma, COPD or cystic fibrosis 2) organisms a) Aspergillus fumigatus (most common) b) Candida albicans c) Aspergillus terreus d) Curvularia lunata e) Helminthosporium species f) Stemphyllium lanuginosum g) Bipolaris hawaiiensis Pathology: - obstructive lung disease caused by an allergic reaction to Aspergillus in the lower airway - impaired mucociliary clearance - destruction of pulmonary parenchyma - bronchiectasis - pulmonary fibrosis Clinical manifestations: 1) persistent, severe, steroid-dependent asthma 2) proximal, segmental bronchiectasis 3) fever 4) expectoration of mucoid plugs, brown sputum 5) may be overlooked until onset of more advanced disease Diagnosis: 1) Major criteria: a) asthma b) blood eosinophilia (> 1000/mm3) c) immediate skin reactivity (Type-I, IgE-mediated) to Aspergillus antigen d) IgG antibodies (Type-III) to Aspergillus antigen e) high serum IgE (> 1000 ng/mL) f) transient or fixed pulmonary infiltrates g) central (proximal) bronchiectasis 2) Minor criteria: a) presence of Aspergillus in sputum b) expectoration of brownish mucus plugs c) late phase (Arthus, type IV) skin-reactivity to Aspergillus antigen Laboratory: 1) complete blood count (CBC): eosinophilia 2) sputum examination: (expectorated mucoid plugs) a) eosinophils b) Aspergillus mycelia 3) antibodies to Aspergillus a) Aspergillus IgE in serum b) Aspergillus IgG in serum 4) increased serum IgE a) normal level virtually excludes diagnosis of allergic bronchopulmonary aspergillosis b) serum IgE can be used to assess the therapeutic response to prednisone Special laboratory: - pulmonary function testing - consistent with obstructive lung disease not responsive to bronchodilator - skin test for IgE to Aspergillus Radiology: 1) pulmonary infiltrates a) especially in upper lobes b) may be transient (recurrent) or fixed 2) atelectasis 3) central bronchiectasis 4) 'gloved finger sign' 5) 'tramtrack line' 6) 'toothpaste shadows' * images CT of thorax [5] Management: 1) glucocorticoids a) systemic glucocorticoids (> 6 months duration) - during an acute phase or exacerbation [2] b) inhaled glucocorticoids may be useful in other circumstances 2) itraconazole or voriconazole may be useful as steroid-sparing agents [2] 3) serum IgE may useful for following course of disease

General

aspergillosis type 1 hypersensitivity; immediate hypersensitivity (allergy) lung disease

Database Correlations

OMIM 103920

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 18, 20, 754
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998,2006, 2009, 2012, 2015, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Agarwal R. Allergic bronchopulmonary aspergillosis. Chest. 2009 Mar;135(3):805-26. PMID: 19265090
  4. Bains SN, Judson MA. Allergic bronchopulmonary aspergillosis. Clin Chest Med. 2012 Jun;33(2):265-81. Review. PMID: 22640845
  5. Chen TH, Hollingsworth H. Images in clinical medicine. Allergic bronchopulmonary aspergillosis. N Engl J Med 2008 Aug 9; 359:e7 PMID: 18687635 Free full text http://www.nejm.org/doi/full/10.1056/NEJMicm055764
  6. Patterson K, Strek ME. Allergic bronchopulmonary aspergillosis. Proc Am Thorac Soc 2010 May 14; 7:237. PMID: 20463254
  7. Knutsen AP, Slavin RG. Allergic bronchopulmonary aspergillosis in asthma and cystic fibrosis. Clin Dev Immunol. 2011;2011:843763. Review. PMID: 21603163 Free PMC Article