Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Idiopathic Bronchiolocentric Interstitial Pneumonia ( Bronchiolocentric fibrosis )
- Radiologic Findings
- * CXR
- Ill-defined tiny nodular opacities in both lungs
* Chest CT
- Extensive ill-defined tiny centrilobular nodules in both lungs
- Previous CT findings (2007-6-5) also show similar lesions without definite change of extent
* Differential Diagnosis
- Including diseases, which CT findings show extensive distributed centrilobular nodules in both lungs
Subacute hypersensitivity pneumonitis
Follicular bronchiolitis
* Pathologic Findings (VATS biopsy)
- At low magnification, the small airways were cuffed by an irregular band of lymphocytes and plasma cells (black arrows)
- High power field image(A) shows an interstitial mononuclear inflammatory infiltrate involved the bronchovascular bundle (black arrow) and perivascular bundle (red arrow). The infiltrate consisted of small round lymphocysts and plasma cells.
- At masson’s stain image(B), the centrilobular compartment of the lobule shows fibrosis which was stained purple (black arrows)
- Brief Review
- Bronchiolocentric interstitial pneumonia is a bronchiolocentric process associated with centrilobular and peribronchiolar fibrosis and a patchy distal lymphocytic alveolitis lacking the presence of interstitial granulomas.
Bronchiolocentric interstitial pneumonia is associated with gas exchange abnormalities.
Patients presented with non-specific symtoms (cough and progressive dyspnea), a restrictive pattern at lung function tests, and peribronchovascular interstitial thickening and traction bronchiectasis on HRCT scans. However, the clinical, imaging and lung function tests were not precisely detailed for most cases in the series.
Most disturbing in bronchiolocentric interstitial pneumonia, in contrast to respiratory bronchiolitis associated with interstitial lung disease and hypersensitivity pneumonitis, is its relatively poor prognosis. At approximately 4 years mean follow-up, 33% of the patients had died of disease and 56% of patients had persistent or progressive complaints.
(Comments from webmaster)
This case is somewhat difficult.
However we would like to introduce the disease entity to the applicants wildly.
Bronchiolocentric fibrosis is a relatively, recently classified disease category and is not familiar to the radiologist.
- References
- 1. Yousem SA, Dacic S. Idiopathic bronchiolocentric interstitial pneumonia.Mod Pathol. 2002 Nov;15(11):1148-53
2. Cordier JF. Challenges in pulmonary fibrosis. 2: Bronchiolocentric fibrosis.Thorax. 2007 Jul;62(7):638-49. Review.
- Keywords
- Lung, Interstitium, Interstitial lung disease, ILD,