bronchocentric granulomatosis

Bronchocentric granulomatosis is a rare chronic condition where airways granulomas form in response to different insults. It is included in the spectrum of eosinophilic lung disease.

Epidemiology

It can affect a wide age spectrum of patients but is thought to peak between the 4 to 7 decades .

Pathology

Its underlying cause is often unclear.

Microscopically, it is characterized by necrotizing granulomatous inflammation of bronchial and bronchiolar epithelium with chronic inflammatory changes in the surrounding lung parenchyma. It does not invade the pulmonary arteries (c.f. necrotizing sarcoid granulomatosis, granulomatosis with polyangiitis (GPA), lymphomatoid granulomatosis) .

The current pathogenetic mechanism is considered to be an immunologic reaction against endobronchial antigens .

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 .

Other rare reported associations include:

• rheumatoid arthritis - see - pulmonary manifestations of rheumatoid arthritis 

Radiographic features

CT 

CT features of bronchocentric granulomatosis are non-specific and can include a focal mass or lobar consolidation with atelectasis .

Treatment and prognosis

It is usually treated with short-term corticosteroids and tends to have a favorable overall prognosis . Some case may resolve spontaneously .

History and etymology

It is thought to have been initially described by Liebow et al. in 1973 .

Siehe auch:

• aspergillus
• pulmonale Eosinophilie

und weiter:

• pulmonale Vaskulitis