Diffuse panbronchiolitis: diagnosis and distinction from various pulmonary diseases with centrilobular interstitial
M Iwata1, T V Colby, M Kitaichi
1Department of Pathology, Mayo Clinic, Rochester, MN.
Abstract:
Diffuse panbronchiolitis (DPB) is a clinicopathologic entity characterized histologically by chronic inflammation principally affecting the respiratory bronchioles. Few pathologists outside Japan are familiar with this entity. The most distinctive pathologic feature of DPB is chronic inflammation and an accumulation of foam cells in the walls of the respiratory bronchioles, adjacent alveolar ducts, and alveoli (PB unit lesion). The differential diagnosis is important both clinically and histologically because of the similarity of DPB to other chronic airway diseases. We report seven cases of DPB and 20 examples of a histologically similar lesion (PB-like lesion) found in a large review of cases of bronchiolitis, cystic fibrosis, bronchiectasis, aspiration pneumonia, extrinsic allergic alveolitis. Wegener's granulomatosis, bronchocentric granulomatosis, and malignant lymphoma. The results indicate that the PB-like lesion is a nonspecific histologic finding; the diagnosis of DPB can be made only in the appropriate clinical setting and when other conditions have been carefully ruled out.
Insights
Diffuse panbronchiolitis (DPB) is a rare airway disease characterized by inflammation in respiratory bronchioles. Distinguishing DPB from similar conditions requires careful clinical and histological evaluation to ensure accurate diagnosis.
Area of Science:
- Pulmonology
- Pathology
- Respiratory Medicine
Background:
- Diffuse panbronchiolitis (DPB) is a clinicopathologic entity primarily affecting respiratory bronchioles.
- It is characterized by chronic inflammation and foam cell accumulation within the respiratory bronchiolar walls and adjacent airspaces, termed the PB unit lesion.
- Familiarity with DPB and its distinctive pathology is limited among pathologists outside Japan.
Purpose of the Study:
- To describe the clinicopathologic features of Diffuse Panbronchiolitis (DPB).
- To differentiate DPB from histologically similar lesions (PB-like lesions) observed in various lung diseases.
- To emphasize the importance of clinical context in diagnosing DPB.
Main Methods:
- Histological examination of seven cases of DPB.
- Review of 20 cases exhibiting PB-like lesions in conditions including bronchiolitis, cystic fibrosis, bronchiectasis, and others.
- Comparative analysis of histological findings between DPB and PB-like lesions.
Main Results:
- The study identified a distinctive PB unit lesion in DPB, involving chronic inflammation and foam cells in respiratory bronchioles.
- Histologically similar PB-like lesions were found in a variety of other pulmonary diseases.
- PB-like lesions were determined to be nonspecific histologic findings.
Conclusions:
- The diagnosis of Diffuse Panbronchiolitis requires integration of clinical findings with histological evidence.
- Histological similarity to DPB can be observed in other chronic airway and lung diseases.
- Careful exclusion of alternative conditions is crucial for a definitive DPB diagnosis.
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