Bronchiolitis obliterans
Obliterative bronchiolitis (OB), also known as bronchiolitis obliterans or constrictive bronchiolitis, is a type of bronchiolitis and refers to bronchiolar inflammation with submucosal peribronchial fibrosis associated with luminal stenosis and occlusions. OB should not be confused with bronchiolitis obliterans organizing pneumonia (BOOP).
Epidemiology
Associations
Recognized associations include:
- inflammatory bowel disease
- rheumatoid arthritis: considered the commonest connective tissue disease to be associated with obliterative bronchiolitis
- medications, e.g. penicillamine
- as a complication following lung transplantation: post lung transplant bronchiolitis obliterans ; can occur in up to 10% of patients, usually within 6-12 months following bone marrow transplantation
- Swyer-James syndrome
Clinical presentation
Its cardinal features are progressive breathlessness and a dry cough .
Pathology
The condition is characterized by concentric luminal narrowing of the membranous and respiratory bronchioles as a result of submucosal and peribronchiolar inflammation and fibrosis without any intraluminal granulation tissue or polyps/polyposis. There is an absence of diffuse parenchymal inflammation.
Etiology
It can result from number of causes:
- idiopathic/cryptogenic
- post-infectious
- post viral (e.g. adenovirus)
- post atypical infection (e.g. Mycoplasma pneumonia)
- noxious fume inhalation
- neuroendocrine hyperplasia (pulmonary tumourlets)
- post-transplant patients
- heart/lung transplants
- representing the obstructive form of chronic lung allograft dysfunction (CLAD)
- hematopoietic stem cell transplants
- heart/lung transplants
Mnemonic: CRITTS
Radiographic features
Plain radiograph
Chest radiographic findings can be normal or, if abnormal, non-specific. Some associated features include:
- hyperinflation
- attenuation of vascular markings
- occasionally reticular/reticulonodular markings
CT
On HRCT chest, there are often sharply defined, areas of decreased lung attenuation associated with vessels of reduced caliber. These changes represent a combination of air trapping and oligemia. This combination can give a mosaic attenuation pattern. Other features include:
History and etymology
It was first described by the French physician AC Reynaud in 1835 .
Differential diagnosis
Consider:
- asthma
- panlobular emphysema: e.g. alpha-1-antitrypsin deficiency
- usually has a lower lung zone predominance
- pulmonary hypertension
- pulmonary trunk enlargement
Siehe auch:
- Rheumatoide Arthritis
- Sarkoidose
- mosaikartige Verdichtungen der Lunge
- Kryptogene organisierende Pneumonie (COP)
- Bronchiolitis
- chronic eosinophilic pneumonia
- In Situ Adenokarzinom der Lunge
- pulmonale Vaskulitis
- Lymphom pulmonale Manifestation
- obliterative bronchiolitis (mnemonic)
- acute fibrinous organizing pneumonia
- bilateral airspace opacities