Diffuse Panbronchiolitis

Definition

Definition of Diffuse Panbronchiolitis
Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, largely restricted to Japan, that is characterized by progressive suppurative and obstructive airway disease, which, if left untreated, progresses to bronchiectasis, respiratory failure, and death.

Symptoms

Symptoms of Diffuse Panbronchiolitis
Typically, symptoms of diffuse panbronchiolitis occur during the second to fifth decade of life and include:

1. Cough
2. Large amounts of sputum (up to 50 milliliters per day in some patients)
3. Dyspnea with minimal activity
4. Wheezing

Causes

Causes of Diffuse Panbronchiolitis
It has no known cause and primarily affects the bronchioles, the smallest airways of the lungs, causing severe, progressive inflammation and obstruction. Left untreated, diffuse panbronchiolitis leads to bronchiectasis, respiratory failure and eventually, death.

Diagnosis

Diagnosis of Diffuse Panbronchiolitis
More than 80 percent of patients who suffer from diffuse panbronchiolitis have a long-term history of sinusitis. This is important when making a diagnosis, which may also include the following tests:

1. Chest X-ray
2. Chest CT scan
3. Pulmonary function tests
4. Sputum culture

Treatment

Treatment of Diffuse Panbronchiolitis
Historically, treatment with medications such as bronchodilators and corticosteroids did little to help avoid the diseases' potentially fatal outcome. Since then, the following macrolide antibiotics have proven effective for a period of up to two years:

1. Low doses of erythromycin
2. Clarithromycin or roxithromycin (when erythromycin is ineffective)

Prognosis

Prognosis of Diffuse Panbronchiolitis
Prior to the discovery that erythromycin helped successfully treat the disease, the prognosis of diffuse panbronchiolitis was poor. To date, long-term treatment with erythromycin has been found to increase the 10-year survival rate of the disease to greater than 90 percent.

Prevention

Prevention of Diffuse Panbronchiolitis
Consult with your doctor.