FlashPath - Lung - Diffuse Panbronchiolitis
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Diffuse panbronchiolitis is a rare lung disease that mainly affects the Japanese. It is characterized by productive cough, dyspnea, and often a history of chronic sinusitis. If left untreated, it can lead to bronchiectasis and respiratory failure. Microscopically, there is a dense inflammatory infiltrate rich in macrophages around the small bronchioles and alveolar walls. Treatment with macrolide antibiotics is effective in slowing the disease course. The disease must be differentiated from other obstructive lung conditions such as chronic bronchitis, bronchiectasis, and asthma.
FlashPath - Lung - Diffuse Panbronchiolitis
- 1. FLASHPATH H A Z E M A L I
- 2. DIFFUSE PANBRONCHIOLITIS H A Z E M A L I
- 3. CLINICAL Diffuse Panbronchiolitis is considered one of the “obstructive lung diseases” that include: • Chronic bronchitis • Emphysema • Bronchiectasis • Asthma
- 4. CLINICAL Obstructive airway disease Restrictive airway disease General features Increase in resistance to airflow due to obstruction at any level Reduced expansion of lung parenchyma Total lung capacity (TLC) Increased Reduced Forced Expiratory Volume in one second (FEV1) Reduced Normal
- 5. CLINICAL • Diffuse panbronchiolits is rare disease of unknown etiology • Almost exclusively in Japanese • Associated with HLA BW54 • Presents with productive cough, dyspnea, and often with history of chronic sinusitis • Often complicated by Infections (e.g. Pseudomonas, H. influenza) • Antibiotics are effective in slowing the course of the disease • Especially macrolides (erythromycin) • If untreated bronchiectasis and respiratory failure
- 6. GROSS • Early cases: – Diffuse, numerous, small yellow nodules (4 mm) • Advanced cases (untreated): – Bronchiectasis
- 7. MICROSCOPY • Dense, bronchiolo-centric chronic inflammatory infiltrate – Distribution: interstitial • small bronchioles and surrounding alveolar walls – Characteristic: rich in foamy macrophages • Acute / organizing pneumonia may be seen • Advanced cases (untreated) – Bronchiectasis – Bronchiolar fibrosis (constrictive bronchiolitis)
- 8. SPECIAL STUDIES • Culture and sensitivity: – Haemophilus influenza and Pseudomonas aeruginosa • Positive Cold hemagglutinins (some cases) – but Mycoplasma antibodies are generally lacking • Positive Rheumatoid factor (some cases) – but few patients with diffuse panbronchiolitis have overt RA
- 9. DIFFERENTIAL DIAGNOSIS Chronic bronchitis Bronchiectasis Asthma Small-airway disease “bronchiolitis” Emphysema Site L a r g e a i r w a y s ( B r o n c h i ) Bronchioles Alveoli Major pathology • Mucous gland hyperplasia • Excess mucus • Inflammation • Airway dilation & scarring • Thickened basement membrane • Smooth muscle hyperplasia • Excess mucus • Inflammation (eosinophils) • Inflammatory scarring & obliteration • Airspace enlargement • Wall destruction • No fibrosis Other obstructive lung diseases:
- 10. DIFFERENTIAL DIAGNOSIS Also: • Connective tissue diseases (especially rheumatoid arthritis) • Mycoplasma infection Clinical correlation is recommended
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