Diffuse panbronchiolitis

Diffuse Panbronchiolitis (DPB) is a chronic inflammatory lung disease primarily affecting the bronchioles. It is characterized by chronic sinusitis, productive cough, and dyspnea. The condition is most commonly observed in individuals of East Asian descent, particularly in Japan and Korea.

Epidemiology

Diffuse Panbronchiolitis is predominantly seen in East Asian populations, with a higher prevalence in Japan. The disease is rare in other ethnic groups. The exact prevalence is not well-documented, but it is considered a rare disease outside of Asia.

Etiology

The exact cause of Diffuse Panbronchiolitis is unknown. However, there is a strong association with certain genetic markers, particularly the HLA-B54 antigen, suggesting a genetic predisposition. Environmental factors have not been clearly identified.

Pathophysiology

The disease is characterized by chronic inflammation of the bronchioles, leading to the formation of lymphoid follicles and infiltration of neutrophils and lymphocytes. This results in obstruction of the small airways and impaired mucociliary clearance, contributing to chronic infection and bronchiectasis.

Clinical Features

Patients with Diffuse Panbronchiolitis typically present with:

• Chronic cough
• Sputum production
• Dyspnea on exertion
• Chronic sinusitis

As the disease progresses, patients may develop respiratory failure and cor pulmonale.

Diagnosis

The diagnosis of Diffuse Panbronchiolitis is based on clinical evaluation, imaging studies, and sometimes lung biopsy.

Imaging

A CT scan of the chest typically shows diffuse nodular opacities and bronchiectasis, particularly in the lower lobes.

Biopsy

A lung biopsy may be performed to confirm the diagnosis, showing characteristic histopathological features such as lymphoid hyperplasia and inflammatory cell infiltration.

Treatment

The mainstay of treatment for Diffuse Panbronchiolitis is long-term macrolide antibiotic therapy, such as erythromycin or clarithromycin. These antibiotics have anti-inflammatory properties that help reduce airway inflammation and improve symptoms.

Prognosis

With appropriate treatment, the prognosis for patients with Diffuse Panbronchiolitis has improved significantly. Long-term macrolide therapy can lead to stabilization or improvement of lung function and symptoms.

See Also

• Bronchiectasis
• Chronic obstructive pulmonary disease
• Interstitial lung disease

External Links

• WikiMD: Diffuse Panbronchiolitis