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].
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.
Treatment
The primary goal of ABPA treatment is to control inflammation and prevent lung damage. Corticosteroids, such as prednisone, are the mainstay of treatment and help reduce inflammation. Antifungal medications, like itraconazole, may be used to decrease the fungal load. In some cases, biologic agents targeting specific immune pathways are considered. Regular monitoring and follow-up are crucial to adjust treatment and manage any side effects.
Prognosis
With appropriate treatment, many patients with ABPA can achieve good control of their symptoms and prevent further lung damage. However, if left untreated, ABPA can lead to progressive lung disease, including bronchiectasis and pulmonary fibrosis. Early diagnosis and intervention are key to improving long-term outcomes.
Etiology
ABPA is caused by an exaggerated immune response to the Aspergillus fungus, which is commonly found in soil, decaying vegetation, and dust. The exact mechanism triggering this hypersensitivity reaction is not fully understood, but it involves a complex interplay between genetic predisposition and environmental exposure.
Epidemiology
ABPA is relatively rare, affecting approximately 1-2% of individuals with asthma and 2-15% of those with cystic fibrosis. It is more common in regions with high humidity and warm climates, where Aspergillus is more prevalent. The condition can occur at any age but is most frequently diagnosed in young adults.
Pathophysiology
In ABPA, the immune system overreacts to Aspergillus antigens, leading to inflammation and damage in the airways. This response involves both immediate hypersensitivity (IgE-mediated) and delayed hypersensitivity (cell-mediated) reactions. The resulting inflammation can cause airway obstruction, mucus plugging, and structural changes in the lungs, such as bronchiectasis.
Prevention
Preventing ABPA involves minimizing exposure to Aspergillus, especially for individuals with asthma or cystic fibrosis. This can include measures such as avoiding environments with high levels of dust or mold, using air purifiers, and maintaining good indoor air quality. Regular monitoring and early intervention in at-risk individuals can also help prevent the development or progression of ABPA.
Summary
Allergic Bronchopulmonary Aspergillosis is a hypersensitivity reaction to the Aspergillus fungus, primarily affecting individuals with asthma or cystic fibrosis. It presents with respiratory symptoms and requires a combination of clinical, laboratory, and imaging studies for diagnosis. Treatment focuses on controlling inflammation and preventing lung damage, with corticosteroids and antifungal medications playing key roles. Early diagnosis and management are crucial for a favorable prognosis.
Patient Information
If you have asthma or cystic fibrosis and experience worsening respiratory symptoms, it is important to consider the possibility of ABPA. This condition is caused by an allergic reaction to a common environmental fungus, Aspergillus. Symptoms can include wheezing, coughing, and shortness of breath. Diagnosis involves specific tests, and treatment typically includes medications to reduce inflammation and control the fungal infection. With proper management, most patients can achieve good control of their symptoms and prevent further lung damage.
References
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- Knutsen AP, Slavin RG. Allergic Bronchopulmonary Aspergillosis in Asthma and Cystic Fibrosis. Clin Dev Immunol. 2011;2011:843763.
- Shah A, Panjabi C. Allergic Bronchopulmonary Aspergillosis: A Perplexing Clinical Entity. Allergy Asthma Immunol Res. 2016;8(4):282-297.
- 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.
- Murray PR, Rosenthal KS, Pfaller MA. Medical Microbiology. Seventh edition. Philadelphia: Elsevier/Saunders; 2013.