Bronchocentric granulomatosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Nikhila Palle, M.B.B.S
Overview
Bronchocentric granulomatosis is not a disease per se but a histopathological finding of the bronchi and bronchioles that is believed to be a nonspecific response to airway injury. It is commonly seen in association with asthma and allergic bronchopulmonary aspergillosis, but can also be observed in non-asthmatics with no identifiable etiological agent.[1]
Historical Perspective
- Bronchocentric granulomatosis was first discovered by Liebow, in 1973.[2]
Classification
- There are two patterns observed in Bronchocentric granulomatosis, the more common pattern is observed in young men with asthma and less common pattern is seen in older patients who are non-asthmatic. [1]
Pathophysiology
- The exact pathophysiology of Bronchocentric granulomatosis is not known.
- On microscopic histopathological analysis, there is central necrotizing granulomatous inflammation of the bronchi and bronchioles.[1]
Causes
- Bronchocentric granulomatosis can be idiopathic or caused by other diseases including asthma and allergic bronchopulmonary aspergillosis. [3]
Differentiating Bronchocentric granulomatosis from other Diseases
- The differential diagnosis of Bronchocentric granulomatosis has to be made from other granulomatous lung diseases and can be divided into infectious and noninfectious diseases:
- Some infectious causes include:
- Mycobacteria i.e. mycobacterium tuberculosis
- Fungi i.e Histoplasma
- Parasites i.e Dirofilaria
- A few noninfectious diseases include:
- Sarcoidosis
- Hypersensitive pneumonitis
- Aspiration pneumonia [4]
Epidemiology and Demographics
- The incidence and prevalence of Bronchocentric granulomatosis is not known.
Age
- Patients of all age groups may develop Bronchocentric granulomatosis.
Gender
- Bronchocentric granulomatosis affects men and women equally.
Race
- There is no racial predilection for Bronchocentric granulomatosis.
Diagnosis
Symptoms
- Symptoms of Bronchocentric granulomatosis may include the following:
- Cough
- Wheezing
- Fever
- Dyspnea
- Blood eosinophilia
Laboratory Findings
- There are no specific laboratory findings associated with Bronchocentric granulomatosis.
- Since around half of the cases of Bronchocentric granulomatosis are associated with allergic bronchopulmonary aspergillosis, Serum IgE levels could be measured as a nonspecific test which may indicate a suspicion towards ABPA. [5]
Imaging Findings
- CT is the imaging modality of choice for Bronchocentric granulomatosis.
- On CT, Bronchocentric granulomatosis is characterized by focal mass or lobar consolidation with atelectasis.[6]
Other Diagnostic Studies
- Bronchocentric granulomatosis may also be diagnosed using open lung biopsy.
- Findings on open lung biopsy include necrotizing granulomatous inflammation of the bronchi and bronchioles with presence of lymphocytes, macrophages and neutrophils.[7]
- Bronchoalveolar lavage can show underlying fungal, bacterial or neoplastic cells that may be present. [7]
Treatment
Medical Therapy
- The mainstay of therapy for Bronchocentric granulomatosis is corticosteroids. An anti fungal agent should be given if there is an underlying fungal infection.[3][8]
Prevention
- There are no primary preventive measures available for Bronchocentric granulomatoiss.
References
- ↑ 1.0 1.1 1.2 Katzenstein AL, Liebow AA, Friedman PJ (1975). "Bronchocentric granulomatosis, mucoid impaction, and hypersensitivity reactions to fungi". Am Rev Respir Dis. 111 (4): 497–537. doi:10.1164/arrd.1975.111.4.497. PMID 1092235.
- ↑ Liebow AA (1973). "The J. Burns Amberson lecture--pulmonary angiitis and granulomatosis". Am Rev Respir Dis. 108 (1): 1–18. doi:10.1164/arrd.1973.108.1.1. PMID 4577269.
- ↑ 3.0 3.1 Koss MN, Robinson RG, Hochholzer L (1981). "Bronchocentric granulomatosis". Hum Pathol. 12 (7): 632–8. PMID 7275101.
- ↑ Mukhopadhyay S, Gal AA (2010). "Granulomatous lung disease: an approach to the differential diagnosis". Arch Pathol Lab Med. 134 (5): 667–90. doi:10.1043/1543-2165-134.5.667. PMID 20441499.
- ↑ Goodman DH, Sacca JD (1977). "Pulmonary cavitation, allergic aspergillosis, asthma and bronchocentric granulomatosis". Chest. 72 (3): 368–9. PMID 891291.
- ↑ Ward S, Heyneman LE, Flint JD, Leung AN, Kazerooni EA, Müller NL (2000). "Bronchocentric granulomatosis: computed tomographic findings in five patients". Clin Radiol. 55 (4): 296–300. doi:10.1053/crad.1999.0380. PMID 10767190.
- ↑ 7.0 7.1 Santos, José Wellington Alves dos; Mello, Carlos Renato; Michel, Gustavo Trindade; Figueiredo, Claudius Wladimir Cornelius de; Miletho, Jader; Zordan, Alessandro (2003). "Granulomatose broncocêntrica idiopática em jovem não asmática". Jornal de Pneumologia. 29 (6): 391–394. doi:10.1590/S0102-35862003000600012. ISSN 0102-3586.
- ↑ Jelihovsky T (1983). "The structure of bronchial plugs in mucoid impaction, bronchocentric granulomatosis and asthma". Histopathology. 7 (2): 153–67. PMID 6852779.