Bronchocentric granulomatosis is a rare chronic condition where airway granulomas form in response to different insults. It is included in the spectrum of eosinophilic lung disease.
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Epidemiology
Bronchocentric granulomatosis can affect a wide age spectrum of patients but is thought to peak between the 4th to 7th decades 6.
Associations
Approximately one-third to half of the affected patients have tissue eosinophilia and tend to have a combination of asthma, peripheral eosinophilia, fungal hyphae at biopsy, and positive sputum cultures for Aspergillus organisms 9.
Other rare reported associations include:
Pathology
Its underlying cause is often unclear. The pathogenetic mechanism is considered to be an immunologic reaction against endobronchial antigens 3.
Microscopic appearance
Microscopically, it is characterised by necrotising granulomatous inflammation of bronchial and bronchiolar epithelium with chronic inflammatory changes in the surrounding lung parenchyma. It does not invade the pulmonary arteries (cf. necrotising sarcoid granulomatosis, granulomatosis with polyangiitis (GPA), lymphomatoid granulomatosis) 9.
Radiographic features
CT
CT features of bronchocentric granulomatosis are non-specific and can include a focal mass or lobar consolidation with atelectasis 9.
Treatment and prognosis
It is usually treated with short-term corticosteroids and tends to have a favourable overall prognosis 4. Some case may resolve spontaneously 6.
History and etymology
It is thought to have been initially described by Liebow et al. in 1973 6.