Bronchocentric granulomatosis is a rare disease characterized by necrotizing granulomatous inflammation beginning in and centering around the bronchiolar walls. The inciting inflammatory precipitant is often unclear. It is associated with numerous diseases of infectious and autoimmune etiologies. These include rheumatoid arthritis; viral, fungal, and mycobacterial infections; diabetes insipidus; ankylosing spondylitis; and systemic granulomatous diseases. Bronchogenic carcinoma has also been described in association with bronchocentric granulomatosis.
Symptoms include fever, cough, wheezing, dyspnea, and blood eosinophilia. Approximately half of patients are asthmatic, and the disease can be associated with Aspergillus fumigatus hypersensitivity or infection.
Related topics: Aspergillosis, Allergic bronchopulmonary aspergillosis
Bronchocentric granulomatosis
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Synopsis

Codes
ICD10CM:
J98.09 – Other diseases of bronchus, not elsewhere classified
SNOMEDCT:
111901001 – Bronchocentric granulomatosis
J98.09 – Other diseases of bronchus, not elsewhere classified
SNOMEDCT:
111901001 – Bronchocentric granulomatosis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Organizing pneumonia
- Allergic bronchopulmonary aspergillosis – biopsy will show eosinophilic infiltration
- Follicular bronchiolitis
- Immunoglobulin G4 (IgG4)-related disease – will see plasma cell infiltration with positive IgG4 immunostaining
- Lymphomatoid granulomatosis
- Vasculitis
- Granulomatosis with polyangiitis (Wegener granulomatosis)
- Necrotizing sarcoid granulomata
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Last Reviewed:06/12/2019
Last Updated:06/16/2019
Last Updated:06/16/2019