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Clinical manifestations and diagnosis of allergic bronchopulmonary aspergillosis

Authors
Praveen Akuthota, MD
Peter F Weller, MD, FACP
Section Editors
Carol A Kauffman, MD
Peter J Barnes, DM, DSc, FRCP, FRS
Deputy Editor
Helen Hollingsworth, MD

INTRODUCTION

Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction in response to colonization of the airways with Aspergillus fumigatus that occurs almost exclusively in patients with asthma or cystic fibrosis (CF) [1-4]. In chronic cases, repeated episodes of bronchial obstruction, inflammation, and mucoid impaction can lead to bronchiectasis, fibrosis, and respiratory compromise [5].

The pathophysiology, diagnosis, and treatment of ABPA will be reviewed here. General issues related to bronchiectasis are discussed separately. (See "Clinical manifestations and diagnosis of bronchiectasis in adults" and "Treatment of bronchiectasis in adults".)

EPIDEMIOLOGY

The prevalence of allergic bronchopulmonary aspergillosis (ABPA) among patients with persistent asthma is estimated at 1 to 2 percent, although rates up to 28 percent have been reported [1,4,6]. Reported rates are higher in patients seen in asthma clinics and those admitted to the hospital with an asthma exacerbation [4]. Among patients with cystic fibrosis, reported prevalences range from 2 to 9 percent [1,5,7-10].

Rarely, ABPA occurs in patients with bronchiectasis, chronic granulomatous disease, hyperimmunoglobulinemia E, and in lung transplant recipients [4,11-13].

PATHOLOGY AND PATHOGENESIS

Allergic bronchopulmonary aspergillosis (ABPA) is characterized pathologically by mucoid impaction of the bronchi, eosinophilic pneumonia, and bronchocentric granulomatosis in addition to the histologic features of asthma [5,14]. Areas of eosinophilic pneumonia are occasionally found, although not a major feature of the disease [15]. Septated hyphae with acute dichotomous branching may be seen in the mucus-filled bronchial lumen, but fungi do not invade the mucosa. Aspergillus is cultured from the sputum in up to two-thirds of patients with ABPA, but hyphae may not be seen by direct microscopy. (See "Bronchocentric granulomatosis".)

              

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Literature review current through: Jun 2016. | This topic last updated: Dec 11, 2015.
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