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.


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].

respiratoric
Cough
  • This leads to symptoms such as wheezing and coughing. You may cough up a lot of phlegm or brown specks of blood. You may also develop a fever. People with asthma or cystic fibrosis are most at risk of ABPA.[fairview.org]
  • The symptoms are similar to those of asthma: intermittent episodes of feeling unwell, coughing and wheezing. Some patients cough up brown-coloured plugs of mucus. The diagnosis can be made by chest X-ray along with sputum, skin and blood tests.[aspergillus.org.uk]
  • […] relieved by coughing up plugs or very thick mucous.[life-worldwide.org]
  • So the patient has difficulty breathing and usually wheezes or coughs. Many patients with ABPA also run a low-grade fever and lose their appetites.[medical-dictionary.thefreedictionary.com]
  • Asthma medications such as oral corticosteroids open the airways and make it easier to cough and clear out the fungus. The use of this medication depends upon the individual and the severity of ABPA.[aaaai.org]
Hemoptysis
  • Hemoptysis — The coughing up of large amounts of blood. Hemoptysis can occur as a complication of ABPA. Immunoglobulin E (IgE) — A type of protein in blood plasma that acts as an antibody to activate allergic reactions.[medical-dictionary.thefreedictionary.com]
  • 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[orpha.net]
  • Exacerbations mimic the initial (acute) presentation, typically characterized by productive cough, fever, dyspnea, and pulmonary infiltrates, sometimes with chest pain or hemoptysis.[asthma.partners.org]
  • 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[symptoma.com]
  • Clinical feature Symptom occasionally be asymptomatic low-grade fever, wheezing, bronchial hyperreactivity, hemoptysis, or productive cough Expectoration of brownish black mucus plugs (31 to 69%) Physical examination normal or polyphonic wheeze Clubbing[slideshare.net]
Pulmonary Disorder
  • Abstract Allergic bronchopulmonary aspergillosis (ABPA) is an immunologic pulmonary disorder caused by hypersensitivity to Aspergillus fumigatus.[ncbi.nlm.nih.gov]
  • Abstract 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.[ncbi.nlm.nih.gov]
  • Definition allergic pulmonary disorder caused by hypersensitivity to Aspergillusfumigatus1 Occurs in asthma or cystic fibrosis2 result of immune response to Aspergillus colonization of airway and poor clearance of mucus secretions subsequent bronchiectasis[slideshare.net]
  • Kradin RL, Mark EJ (2008) The pathology of pulmonary disorders due to Aspergillus spp. Arch Pathol Lab Med 132: 606-614.[omicsonline.org]
Productive Cough
  • Learn about this topic in these articles: symptoms In aspergillosis allergic bronchopulmonary aspergillosis , seen especially in patients with chronic pulmonary diseases, include a chronic productive cough and purulent sputum occasionally tinged with[britannica.com]
  • Symptoms and signs are those of asthma with the addition of productive cough and, occasionally, fever and anorexia.[msdmanuals.com]
  • 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[orpha.net]
  • Exacerbations mimic the initial (acute) presentation, typically characterized by productive cough, fever, dyspnea, and pulmonary infiltrates, sometimes with chest pain or hemoptysis.[asthma.partners.org]
  • Clinical feature Symptom occasionally be asymptomatic low-grade fever, wheezing, bronchial hyperreactivity, hemoptysis, or productive cough Expectoration of brownish black mucus plugs (31 to 69%) Physical examination normal or polyphonic wheeze Clubbing[slideshare.net]
Rales
  • Focal inspiratory rales suggestive of a pneumonia or area of bronchiectasis may raise one's suspicion and lead to chest radiography. Laboratory evaluation Laboratory studies are required to confirm the diagnosis (Table 2).[asthma.partners.org]
Chronic Productive Cough
  • Learn about this topic in these articles: symptoms In aspergillosis allergic bronchopulmonary aspergillosis , seen especially in patients with chronic pulmonary diseases, include a chronic productive cough and purulent sputum occasionally tinged with[britannica.com]
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  • 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[mountsinai.org]
    • If the disease progresses, symptoms may include: Coughing, sometimes accompanied by mucus or blood Wheezing Fever Chest pain Difficulty breathing Symptoms of invasive aspergillosis may include: Fever Chills Breathing difficulties, such as shortness of[my.clevelandclinic.org]
    • 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[mayoclinic.org]
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  • Course
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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.

    Laboratory

    Serum
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  • Microbiology
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  • Imaging

    X-ray
    Pulmonary Infiltrate
    • A typical steroid dose is prednisone 0.5 mg/kg for approximately 2 weeks or until the pulmonary infiltrates clear.[asthma.partners.org]
    • Episodic bronchospasm, expectoration of mucous plugs, and fleeting pulmonary infiltrates are common manifestations of the disease.[ingentaconnect.com]
    • Clinically, a patient presents with chronic asthma, recurrent pulmonary infiltrates, and bronchiectasis. The population prevalence of ABPA is not clearly known, but the prevalence in asthma clinics is reported to be around 13%.[ncbi.nlm.nih.gov]
    • Abstract 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.[ncbi.nlm.nih.gov]
    • Diagnostic criteria for ABPA include the presence of asthma,a history of pulmonary infiltrates, peripheral blood eosinophilia, immediate-type skin reactivity, serum precipitating antibodies toAspergillus-specific IgE and IgG and central (proximal) bronchiectasis[ucdenver.edu]
    X-Ray Abnormal
    • The diagnosis of ABPA requires the presence of a constellation of symptoms, x-ray abnormalities and investigation results that provide evidence of the presence of sensitisation to Aspergillus as well as a ‘response’ by the body to the fungus.[lungfoundation.com.au]
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  • Test Results

    Pulmonary Function Test
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  • Treatment

    Prognosis

    Complications

    Asthma
    • Pennington, MD, Pulmonologist, Wake Forest School of Medicine Click here for Patient Education Asthma and Related Disorders Asthma Drug Treatment of Asthma Treatment of Acute Asthma Exacerbations Allergic Bronchopulmonary Aspergillosis (ABPA) Allergic[msdmanuals.com]
    • […] symptoms, as measured by the Asthma Control Test (ACT).[dovepress.com]
    • Poorly-controlled asthma is a common finding, with a case series only finding 19% of ABPA patients with well-controlled asthma.[en.wikipedia.org]
    • The corticosteroid-dependent asthma stage may have recurrent exacerbations of allergic bronchopulmonary aspergillosis and severe asthma.[annals.org]
    • Ann Allergy Asthma Immunol. 2005;95:488-493.[consultantlive.com]
    Bronchiectasis
    • For a discussion of the differential diagnosis of bronchiectasis please refer to the article bronchiectasis and more specifically central bronchiectasis .[radiopaedia.org]
    • Imaging: The CXR shows bronchial wall thickening and impressive central bronchiectasis. The CT demonstrates varicoid and cystic central bronchiectasis in all 5 lobes and mucous plugging.[ucdenver.edu]
    • The disorder needs to be detected before bronchiectasis has developed because the occurrence of bronchiectasis is associated with poorer outcomes.[ncbi.nlm.nih.gov]
    • Bronchiectasis — A disorder of the bronchial tubes marked by abnormal stretching, enlargement, or destruction of the walls. Bronchiectasis is usually caused by recurrent inflammation of the airway and is a diagnostic criterion of ABPA.[medical-dictionary.thefreedictionary.com]
    • […] of the proximal bronchi ("central bronchiectasis").[asthma.partners.org]
    Extrinsic Allergic Alveolitis
    • See separate Extrinsic Allergic Alveolitis article. Pathophysiology In a healthy, immunocompetent individual, macrophages and neutrophils normally defend against the inhaled fungus.[patient.info]
    Recurrent Pneumonia
    • Other patients will be diagnosed in the process of evaluating severe, steroid-dependent asthma or recurrent pneumonias in an asthmatic patient. Physical examination is generally non-specific.[asthma.partners.org]
    Eosinophilia
    • Eosinophilia is usually also present.[medical-dictionary.thefreedictionary.com]
    • All children tested had positive type 1 immediate hypersensitivity to skin tests for A fumigatus , in sputum eosinophilia, and Aspergillus cultured from sputum.[pediatrics.aappublications.org]
    • Peripheral blood eosinophilia is common. The serum IgE level is characteristically very high, generally greater than 1000 U/ml. Serum precipitins to aspergillus are generally positive in ABPA.[asthma.partners.org]
    • Beware of other causes of blood eosinophilia such as drugs, Churg Strauss, Wegeners, worms and tropical eosinophilia (Peckham et al, 2001 ). References Becker JW, Burke W, McDonald G, et a l.[cfmedicine.com]
    • Markers Laboratory findings include: elevated Aspergillus -specific IgE elevated precipitating IgG against Aspergillus peripheral eosinophilia positive skin test Plain radiograph Early in the disease chest x-rays will appear normal, or only demonstrate[radiopaedia.org]
    Pulmonary Eosinophilia
    • .: Corticosteroid treatment and prognosis in pulmonary eosinophilia . Thorax 1989, 44: 925–929. PubMed CrossRef Google Scholar 38. Judson MA, Stevens DA: Current pharmacotherapy of allergic bronchopulmonary aspergillosis .[link.springer.com]
    • Asthmatic pulmonary eosinophilia: a review of 65 cases. Br J Dis Chest 1977 ;71: 115 - 122 12. Capewell S, Chapman BJ, Alexander F, Greening AP, Crompton GK. Corticosteroid treatment and prognosis in pulmonary eosinophilia.[nejm.org]

    Etiology

    Causes

    Fungus
    • Aspergillus is a common fungus. It is often found in soil, on plants, and in rotting vegetation. Tiny particles of the fungus can be breathed into the lungs. This doesn’t cause a problem in most people.[fairview.org]
    • The cloudiness on the left side of this x-ray is caused by the fungus. Causes Aspergillosis is caused by a fungus called aspergillus. The fungus is often found growing on dead leaves, stored grain, compost piles, or in other decaying vegetation.[mountsinai.org]
    • This helps remove the fungus from your airways. Treating the Fungus Your doctor might have you take an antifungal medication, such as itraconazole, to get rid of as much of the fungus in your airways as possible.[healthline.com]
    • Treatment of ABPA is based around the concepts of either suppressing the airway hypersensitivity response to the fungus and/or trying to reduce the amount of fungus present in the airways thereby reducing the ‘stimulus’ to the allergic response.[lungfoundation.com.au]
    • Treatment & Management The fungus that causes a reaction is difficult to avoid, so medication is typically prescribed to manage ABPA.[aaaai.org]

    Epidemiology

    Sex distribution
    Age distribution

    Pathophysiology

    Prevention

    Summary

    Patient Information

    Self-assessment

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    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.

    1. Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis - AP Knutsen, RG Slavin - Clinical Reviews in Allergy and Immunology, 1991 - Springer
    2. A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
    3. A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
    4. A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
    5. 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
    6. Allergic bronchopulmonary aspergillosis - RG Slavin, CC Gottlieb, LV Avioli - Archives of internal medicine, 1986 - Am Med Assoc
    7. A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
    8. Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
    9. A practical workup for eosinophilia - ML Brigden - Postgraduate medicine, 1999 - postgradmed.org
    10. Allergic bronchopulmonary aspergillosis - I Tillie‐Leblond, AB Tonnel - Allergy, 2005 - Wiley Online Library
    11. Allergic bronchopulmonary aspergillosis with low serum immunoglobulin E - RH Schwartz, GE Hollick - Journal of Allergy and Clinical Immunology, 1981 - Elsevier
    12. A review of non-cystic fibrosis pediatric bronchiectasis - EJ Boren, SS Teuber, ME Gershwin - Clinical Reviews in Allergy and , 2008 - Springer
    13. Allergic bronchopulmonary aspergillosis associated with smoking moldy marihuana. - R Llamas, DR Hart - CHEST Journal, 1978 - journal.publications.chestnet.org
    14. A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
    15. Allergic broncho‐pulmonary aspergillosis - DS McCarthy, J Pepys - Clinical & Experimental Allergy, 1971 - Wiley Online Library
    16. A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
    17. Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
    18. Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
    19. 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
    20. Allergic bronchopulmonary aspergillosis - BA Cockrill, MD, CA Hales, MD - Annual review of medicine, 1999 - annualreviews.org
    21. Allergic bronchopulmonary aspergillosis - PA Greenberger - Journal of Allergy and Clinical Immunology, 2002 - Elsevier
    22. A randomized trial of itraconazole in allergic bronchopulmonary aspergillosis - DA Stevens, HJ Schwartz, JY Lee - England Journal of , 2000 - Mass Medical Soc
    23. 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
    24. Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
    25. 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
    26. Adjunctive therapy of allergic bronchopulmonary aspergillosis with itraconazole. - DW Denning, JE Van Wye, NJ Lewiston - , 1991 - chestjournal.chestpubs.org
    27. 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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