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Mucoid Impaction
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Plastic bronchitis is a rare, underdiagnosed and potentially fatal condition. It is characterised by the formation and expectoration of branching gelatinous plugs that assume the shape of the airways. These airway plugs differ from the allergic mucin that characterises allergic bronchopulmonary aspergillosis and mucoid impaction of the bronchi. Plastic bronchitis is most often encountered in the paediatric population following corrective cardiac surgery, such as the Fontan procedure. It also occurs in adults. Plastic bronchitis in adults is rare, heterogeneous in its aetiology, and can lead to respiratory distress or even life-threatening airway obstruction. Plastic bronchitis in adulthood should not be overlooked, particularly in patients with chronic inflammatory lung diseases. This review presents current understanding of the presentation, aetiology, pathogenesis, pathology and management of plastic bronchitis in adults.
Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity syndrome triggered against antigens of Aspergillus fumigatus, a fungus that most commonly colonizes the airways of patients with bronchial asthma and cystic fibrosis. It presents clinically with refractory asthma, hemoptysis and systemic manifestations including fever, malaise and weight loss. Radiologically, it presents with central bronchiectasis and recurrent episodes of mucus plugging. The mucus plugs in ABPA are generally hypodense but in up to 20% of patients the mucus can be hyperdense on computed tomography. This paper reviews the literature on the clinical significance of hyperattenuated mucus in patients with ABPA.
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Allergic bronchopulmonary aspergillosis can be expressed pathologically in response to certain species of the organism as microgranulomatous lesions, mucoid impaction of bronchi, eosinophilic pneumonia, or bronchocentric granulomatosis. These lesions can coexist in various permutations and combinations, but each can also be produced by other agents and mechanisms, only some of which have been identified. The immunologic mechanisms responsible for these various distinctive tissue responses remain to be determined. From analysis of observations in 23 patients it is concluded that bronchocentric granulomatosis represents a distinctive lesion consisting of replacement of bronchial epithelium by palisaded granulation tissue. The bronchial contents and the granulomas thus formed contain an exudate rich in eosinophils that often become conglutinated and partly necrotic. Arteries may show an angiitis, most extensive on the side of the involved bronchi, suggesting that their involvement is incidental. Because the prognosis in bronchocentric granulomatosis is generally favorable, it is important that the lesion be distinguished from the angiocentric granulomatoses. Those patients with bronchocentric granulomatosis in whom noninvasive fungi have been found in proximal bronchi are invariably chronic asthmatics, usually have severe eosinophilia, and sometimes have mucoid impaction of proximal bronchi. In the nonasthmatics, these features are usually absent and no possibly sensitizing agent has been identified, but an allergic pathogenesis also is strongly suspected.
We describe the pathologic features in surgically excised lung tissue specimens from 18 cases of allergic bronchopulmonary aspergillosis (ABPA). The main abnormalities involved bronchi and bronchioles. All cases showed bronchocentric granulomatosis (BCG), mucoid impaction of bronchi (MIB), or both. The impacted mucin of MIB contained large numbers of eosinophils and Charcot-Leyden crystals. A distinctive exudative bronchiolitis was present distal to areas of BCG in 13 cases. This lesion was characterized by filling of bronchiolar lumens with necrotic neutrophils and eosinophils in a basophilic mucinous exudate. A peribronchiolar chronic inflammatory infiltrate was seen in 15 cases; eosinophils were prominent in 10 of these cases. Foci of eosinophilic pneumonia were seen in 13 cases, and noninvasive fungal hyphae were identified in 14. We conclude that the finding of BCG or MIB, or a combination of both, especially in conjunction with tissue eosinophilia, should suggest the diagnosis of ABPA. When noninvasive fungal hyphae are also present, the changes are diagnostic of ABPA or a related allergic fungal reaction.
Aspergillosis is a serious pathologic condition caused by Aspergillus organisms and is frequently seen in immunocompromised patients. At computed tomography (CT), saprophytic aspergillosis (aspergilloma) is characterized by a mass with soft-tissue attenuation within a lung cavity. The mass is typically separated from the cavity wall by an airspace ("air crescent" sign) and is often associated with thickening of the wall and adjacent pleura. CT findings in allergic bronchopulmonary aspergillosis consist primarily of mucoid impaction and bronchiectasis involving predominantly the segmental and subsegmental bronchi of the upper lobes. Aspergillus necrotizing bronchitis may manifest as an endobronchial mass, obstructive pneumonitis or collapse, or a hilar mass. Bronchiolitis is characterized by centrilobular nodules and branching linear or nodular areas of increased attenuation ("tree-in-bud" pattern). Obstructing bronchopulmonary aspergillosis mimics allergic bronchopulmonary aspergillosis at CT and manifests as bilateral bronchial and bronchiolar dilatation, large mucoid impactions, and diffuse lower lobe consolidation caused by postobstructive atelectasis. Characteristic CT findings in angioinvasive aspergillosis consist of nodules surrounded by a halo of ground-glass attenuation ("halo sign") or pleura-based, wedge-shaped areas of consolidation. Although imaging findings in pulmonary aspergillosis may be nonspecific, in the appropriate clinical setting, familiarity with the CT findings may suggest or even help establish the diagnosis.
Diagnostic atlas of non-neoplastic lung disease
- A-L Katzenstein
Non-neoplastic pathology
- S Mukhopadhyay
Specialty imaging - HRCT of the lung
- J W Gurney