Allergic Bronchopulmonary Aspergillosis (ABPA)

Allergic bronchopulmonary aspergillosis is a disease characterized by a hypersensitivity reaction to aspergillus fumigatus after its repeated inhalation and is most frequently encountered in patients suffering from asthma or cystic fibrosis (CF). The diagnosis is based on clinical, radiographic and microbiological criteria, but symptoms may not be apparent until advanced stages of the disease occur.

The disease is related to the following process: infectious.

Presentation

Allergic bronchopulmonary aspergillosis (ABPA) is one of the main forms of pulmonary disease caused by aspergillus fumigatus, perhaps the most important fungal pathogen in clinical practice, together with candida albicans [1] [2] [3]. It is primarily diagnosed in children and younger adults [4], and signs and symptoms stem from a hypersensitivity reaction induced by repeated inhalation of A. fumigatus conidia, after which both innate and adaptive immune mechanisms initiate an inflammatory reaction [1] [4] [5]. ABPA is diagnosed in up to 6% of patients suffering from chronic asthma and in almost 15% of individuals harboring a diagnosis of cystic fibrosis, suggesting that these two conditions are most important risk factors [5] [6]. In the majority of cases, clinical deterioration of preexisting pulmonary disease is the principal manifestation, with symptoms such as more pronounced cough, wheezing, increased sputum production, hemoptysis, dyspnea, chest pain and the appearance of exercise-induced asthma [2] [6]. Recurrent fever can also be reported [6]. In more severe cases, cyanosis, digital clubbing, and cor pulmonale can be present. However, patients often show minimal signs of the disease for a prolonged period of time, especially if neither asthma nor CF is present [6]. Moreover, without an adequate diagnosis and early initiation of therapy, the clinical course of ABPA is distinguished by repeated remission and exacerbation of symptoms, eventually leading to pulmonary fibrosis, bronchiectasis and chronic production of sputum [2] [3]. The importance of early recognition lies in the fact that pulmonary fibrosis has a poor long-term outcome and may progress to respiratory failure [2] [5].

Entire body system
  • Chills Possible Complications Health problems from the disease or treatment include: Amphotericin B can cause kidney damage and unpleasant side effects such as fever and chills Bronchiectasis (permanent scarring and enlargement of the small sacs in the lungs [source] Symptoms of allergic pulmonary aspergillosis may include: Cough Coughing up blood or brownish mucus plugs Fever General ill feeling (malaise) Wheezing Weight loss Other symptoms depend on the part of the body affected, and may include: Bone pain Chest pain Chills [source] Signs and symptoms depend on which organs are affected, but in general, invasive aspergillosis can cause: Fever and chills Cough that brings up blood-streaked sputum (hemoptysis) Severe bleeding from your lungs Shortness of breath Chest or joint pain [source]
  • Constitutional Symptom all the criteria and there is no documented mucoid impaction on CT chest or bronchoscopy 2 Response Clinical improvement (resolution of constitutional symptoms, improvement in asthma control) Major radiological improvement* IgE decline by 25% of baseline [source] staging of ABPA in asthma Stage Definition Features 0 Asymptomatic GINA definition of controlled asthma On investigation fulfils the diagnostic criteria of ABPA (Table 4) Has not been previously diagnosed to have ABPA 1 Acute Patient has uncontrolled asthma/constitutional [source] symptoms Fulfils diagnostic criteria for ABPA Not previously diagnosed to have ABPA 1a With mucoid impaction Meets all the criteria and there is documented mucoid impaction on chest radiograph, CT chest or bronchoscopy 1b Without mucoid impaction Meets [source]
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respiratoric
  • Chronic Cough In the chronic stage, the symptoms are chronic cough, dyspnea, fatigue, anorexia, and weight loss. [source]
  • Chronic Productive Cough Learn about this topic in these articles: symptoms Characteristic symptoms of allergic bronchopulmonary aspergillosis , seen especially in patients with chronic pulmonary diseases, include a chronic, productive cough and purulent sputum occasionally tinged [source]
  • Cough Along with a persistent cough, breathlessness, tiredness and weight loss, coughing up blood is a common symptom. [source] General symptoms can include: shortness of breath a persistent cough coughing up mucus or coughing up blood fatigue weight loss a high temperature Contact your doctor if you develop severe or persistent symptoms of aspergillosis, particularly if you have [source] It usually affects the respiratory system (windpipe, sinuses and lungs) and causes wheezing and coughing, but it can spread to anywhere in the body. [source]
  • Hemoptysis However, it may cause hemoptysis[source] if pulmonary function is adequate Bronchial artery embolization may be used for life-threatening hemoptysis in patients unlikely to tolerate surgery or in patients with recurrent hemoptysis (eg, patients with CF in whom hemoptysis may be related to underlying [source] Aspergilloma is associated with hemoptysis, which may be severe and life threatening. [source]
  • Productive Cough Learn about this topic in these articles: symptoms Characteristic symptoms of allergic bronchopulmonary aspergillosis , seen especially in patients with chronic pulmonary diseases, include a chronic, productive cough and purulent sputum occasionally tinged [source] Symptoms and signs are those of asthma with the addition of productive cough and, occasionally, fever and anorexia. [source] Although affected individuals can occasionally be asymptomatic, most of them present with wheezing, bronchial hyperreactivity, hemoptysis, productive cough, low-grade fever, malaise, weight loss, and/or worsening symptoms of asthma and cystic fibrosis [source]
  • Pulmonary Disorder Allergic bronchopulmonary aspergillosis (ABPA) is an immunological pulmonary disorder caused by hypersensitivity to Aspergillus fumigatus, manifesting with poorly controlled asthma, recurrent pulmonary infiltrates and bronchiectasis. [source] Definitionallergic pulmonary disorder caused by hypersensitivity to Aspergillusfumigatus1Occurs in asthma or cystic fibrosis2result of immune response to Aspergillus colonization of airway and poor clearance of mucus secretionssubsequent bronchiectasis [source] Differential Diagnosis Aspergillus hypersensitive bronchial asthmapulmonary tuberculosis in endemic areascommunity-acquired pneumonia (especially acute presentations)other inflammatory pulmonary disorders eg. eosinophilic pneumonia, bronchocentricgranulomatosis [source]
  • Rales Focal inspiratory rales suggestive of a pneumonia or area of bronchiectasis may raise one's suspicion and lead to chest radiography. [source]
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Workup

The diagnosis of ABPA is not easy to attain in patients who develop nonspecific lung-related signs and symptoms, especially when pulmonary conditions, such as asthma and CF, are concomitantly present. A detailed patient history and a thorough physical examination (with an emphasis on pulmonary auscultation), however, are detrimental parts of the diagnostic workup, as they can identify recent exacerbation or the appearance of new lung-related symptoms. Moreover, many individuals already have some other allergic disorders (for eg. rhinitis, conjunctivitis, atopic dermatitis, etc.), which may be another clue toward ABPA as a differential diagnosis [4]. Because clinical findings are not specific for the diagnosis of ABPA, its recognition relies on the fulfillment of the following criteria [1] [2] [6] [7] [8]:

  • History of asthma (considered to be one of the main prerequisites).
  • Clinical deterioration of preexisting pulmonary symptoms (if patients suffer from asthma or CF).
  • Immediate hypersensitivity to aspergillus spp. confirmed by a skin prick test.
  • Elevated serum immunoglobulin (Ig) E levels (> 416 IU/mL or > 1000 ng/mL).
  • Presence of IgE or IgG-specific antibodies to aspergillus spp.
  • Peripheral blood eosinophilia confirmed on a complete blood count (CBC).
  • Radiographic signs - Plain radiography, often employed as the initial imaging method, shows pulmonary infiltrates and consolidation (also termed non-homogenous opacities), as well as mucus plugs, lobar or segmental lung collapse and presence of fluid in the bronchi in the initial stages of the disease [4] [6]. Inflammation of the airways, often designated as "tramline" sign, is frequently visible in patients suffering from ABPA, whereas other notable radiographic features are edema of the bronchial wall, "toothpaste" shadows, and mucoid plugs causing "glover finger" opacities [4] [6]. Although plain radiography can be highly useful, high-resolution computed tomography (HRCT) is proven to be a superior method for evaluation of many pulmonary diseases, including ABPA, due to its ability to visualize lesions in more detail [1] [4] [5] [6]. For this reason, HRCT should be used whenever possible in patients with suspected ABPA.

Test Results

Pulmonary Function Test

Laboratory

Serum
Microbiology

Imaging

X-ray
  • Pulmonary Infiltrate Allergic Bronchopulmonary Aspergillosis Case Example A 23-year-old man was evaluated for asthma and bilateral pulmonary infiltrates[source] A typical steroid dose is prednisone 0.5 mg/kg for approximately 2 weeks or until the pulmonary infiltrates clear. [source] His chest X-ray ( Figure 1a ) and chest CT scan ( Figure 1b ) revealed bilateral pulmonary infiltrates and central bronchiectasis. [source]
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Treatment

Prognosis

Complications

  • Asthma Clinical deterioration of preexisting pulmonary symptoms (if patients suffer from asthma or CF). [source] However, patients often show minimal signs of the disease for a prolonged period of time, especially if neither asthma nor CF is present. [source] The diagnosis of ABPA is not easy to attain in patients who develop nonspecific lung-related signs and symptoms, especially when pulmonary conditions, such as asthma and CF, are concomitantly present. [source]
  • Bronchiectasis Bronchiectasis alone may be present in patients with asthma not complicated by ABPA but it is uncommon for asthmatics withoutABPA to have severe forms of bronchiectasis involving multiple lobes. [source] Imaging: The CXR shows bronchial wall thickening and impressive central bronchiectasis[source] The CT demonstrates varicoid and cystic central bronchiectasis in all 5 lobes and mucous plugging. [source]
  • Eosinophilia Eosinophilia is usually also present. [source] Lab Eosinophilia, increased IgE, A fumigatus in sputum. [source] Eosinophilia — An abnormal increase in the number of eosinophils in the blood. [source]
  • Extrinsic Allergic Alveolitis Extrinsic allergic alveolitis[source] See separate Extrinsic Allergic Alveolitis article. [source] Another disease caused by Aspergillus spp. is an extrinsic allergic alveolitis known as malt worker's lung. [source]
  • Pulmonary Eosinophilia .: Corticosteroid treatment and prognosis in pulmonary eosinophilia[source] Clinical Staging The spectrum of ABPA varies widely, from individuals with mild asthma and occasional episodes of pulmonary eosinophilia with no long-term sequelae, to patients with fibrosis, honey-comb lung, and respiratory failure. [source]
  • Recurrent Pneumonia Other patients will be diagnosed in the process of evaluating severe, steroid-dependent asthma or recurrent pneumonias in an asthmatic patient. [source]
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Etiology

Causes

  • Fungus Aspergillus is a common fungus[source] You may also be given medicines to help kill the fungus[source] Tiny particles of the fungus can be breathed into the lungs. [source]
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Epidemiology

Sex distribution
Age distribution

Pathophysiology

Prevention

Summary

Patient Information

Self-assessment

References

  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;43(8):850-873.
  2. Greenberger PA, Bush RK, Demain JG, Luong A, Slavin RG, Knutsen AP. Allergic Bronchopulmonary Aspergillosis. J Allergy Clin Immunol Pract. 2014;2(6):703-708.
  3. Mandell GL, Bennett JE, Dolin R. Mandel, Douglas and Bennett's Principles and Practice of Infectious Diseases. 8th ed. Philadelphia, Pennsylvania: Churchill Livingstone; 2015.
  4. Tillie-Leblond I, Tonnel AB. Allergic bronchopulmonary aspergillosis. Allergy. 2005;60(8):1004-1013.
  5. Knutsen AP, Slavin RG. Allergic Bronchopulmonary Aspergillosis in Asthma and Cystic Fibrosis. Clin Dev Immunol. 2011;2011:843763.
  6. Shah A, Panjabi C. Allergic Bronchopulmonary Aspergillosis: A Perplexing Clinical Entity. Allergy Asthma Immunol Res. 2016;8(4):282-297.
  7. Agarwal R, Gupta D, Aggarwal AN, et al. Clinical significance of decline in serum IgE levels in allergic bronchopulmonary aspergillosis. Respir Med. 2010;104(2):204-210.
  8. Murray PR, Rosenthal KS, Pfaller MA. Medical Microbiology. Seventh edition. Philadelphia: Elsevier/Saunders; 2013.

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  • A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
  • A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
  • Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis - AP Knutsen, RG Slavin - Clinical Reviews in Allergy and Immunology, 1991 - Springer
  • A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
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  • Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
  • Allergic bronchopulmonary aspergillosis - I Tillie‐Leblond, AB Tonnel - Allergy, 2005 - Wiley Online Library
  • A practical workup for eosinophilia - ML Brigden - Postgraduate medicine, 1999 - postgradmed.org
  • A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
  • A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
  • In the collagen region of SP-A2 with increased levels of total IgE antibodies and eosinophilia in patients with allergic bronchopulmonary aspergillosis - S Saxena, T Madan, A Shah, K Muralidhar - Journal of allergy and , 2003 - Elsevier
  • Fumigatus allergens in A fumigatus–sensitized asthmatic subjects allows diagnostic separation of allergic bronchopulmonary aspergillosis from fungal - S Hemmann, G Menz, C Ismail, K Blaser - Journal of allergy and , 1999 - Elsevier
  • Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
  • Allergic bronchopulmonary aspergillosis - PA Greenberger - Journal of Allergy and Clinical Immunology, 2002 - Elsevier
  • Allergic bronchopulmonary aspergillosis in cystic fibrosis—state of the art: Cystic Fibrosis Foundation Consensus Conference - DA Stevens, RB Moss, VP Kurup - Clinical Infectious , 2003 - cid.oxfordjournals.org
  • A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
  • Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
  • A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
  • Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
  • Allergic bronchopulmonary aspergillosis with low serum immunoglobulin E - RH Schwartz, GE Hollick - Journal of Allergy and Clinical Immunology, 1981 - Elsevier
  • Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org

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