Allergic Bronchopulmonary Aspergillosis
Joseph J. Maleszewski, M.D.
Marie-Christine Aubry, M.D.
Allen P. Burke, M.D.
Definition
Allergic bronchopulmonary aspergillosis (ABPA) is a term used to describe exacerbation of underlying airway injury, usually in the setting of asthma or cystic fibrosis, by exposure and subsequent hypersensitivity to Aspergillus-associated antigens.
While Aspergillus is far and away the most common offending agent, other fungi (e.g., Candida albicans, Curvularia sp., Helminthosporium sp., Torulopsis glabrata, Saccharomyces cerevisiae, and Trichosporon beigelii) are known to produce antigens that have been associated with similar clinical and pathologic features. Hence, ABPA can be considered more broadly under the term “allergic bronchopulmonary fungal disease.”
Epidemiology
Most patients with ABPA have an underlying chronic airway condition, with asthma being the most common and cystic fibrosis being the second. Hypersensitivity to Aspergillus is a relatively common phenomenon, occurring in ˜25% of those with asthma. ABPA is less common, affecting anywhere between 1% and 13% of asthmatics and ˜10% of those with cystic fibrosis.1,2 Steroid-dependent asthmatics appear to be at higher risk for ABPA than do nonsteroid-dependent asthmatics.
Clinical, Laboratory, and Radiologic Features
Symptoms
Most patients with ABPA present with worsening of their underlying lung disease. Exacerbation of symptoms of asthma, such as increased cough, wheezing, and expectoration of sputum plugs often occurs. Episodic fever and/or hemoptysis may also occur. These symptoms often occur despite aggressive antiasthmatic medications. Over the years, a list of major and minor criteria that combine these symptoms has been created to aide in rendering a diagnosis in those patients with asthma (Table 33.1). Modifications have been made to evaluate other patients (such as those with cystic fibrosis) as well. A subset of patients with ABPA may also have symptoms of chronic sinusitis, which may indicate concurrent allergic fungal sinusitis.
The clinical presentation is divided into five stages (Table 33.2). Stage I is considered the acute phase, characterized by fever, cough, chest pain, hemoptysis, and/or sputum production. Stage II is remission in which the patient is usually asymptomatic or has had stabilization of their asthma. Stage III is exacerbation, where symptoms of the acute phase may return or laboratory/radiologic evidence of the disease is again apparent. In stage IV, there is persistent severe asthma that is corticosteroid dependent. Patients with stage V disease will usually exhibit cyanosis and extreme dyspnea. These end-stage patients will usually also have radiologically or pathologically evident fibrosis.
Serologic and Hypersensitivity Testing
Patients with ABPA will almost invariably have elevated total serum IgE concentration, as well as antibodies (IgE or IgG) directed against fungal organisms (most commonly Aspergillus fumigates). Cutaneous hypersensitivity to fungal antigens is also a common finding, with ABPA patients demonstrating an almost immediate wheal-and-flare reaction.
Microbiology
The most common offending organism in ABPA is Aspergillus fumigates. While it is sometimes possible to culture such from sputum, the finding is neither sensitive nor specific for the diagnosis on its own.
Imaging
Chest radiography can range from normal in the early stages of the disease to central and upper lung zone infiltrates in later phases. Mucoid impaction of the proximal airways may result in band-like opacities
extending from the hilum. Inflamed and thickened bronchi may also result in “ring signs/shadows,” in a similar distribution. High-resolution computed tomography (HRCT) scans offer better assessment of the pattern and distribution. The central distribution of the bronchiectasis is perhaps the most characteristic sign of ABPA, radiologically.6 Additional findings include mosaic attenuation, centrilobular nodules, and tree-in-bud opacities.
extending from the hilum. Inflamed and thickened bronchi may also result in “ring signs/shadows,” in a similar distribution. High-resolution computed tomography (HRCT) scans offer better assessment of the pattern and distribution. The central distribution of the bronchiectasis is perhaps the most characteristic sign of ABPA, radiologically.6 Additional findings include mosaic attenuation, centrilobular nodules, and tree-in-bud opacities.
TABLE 33.1 Diagnostic Criteria for ABPA | |||
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