Diffuse panbronchiolitis (Q42853)

Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease principally affecting the respiratory bronchioles, causing a progressive suppurative and severe obstructive respiratory disorder. Onset occurs in the second to fifth decade of life and manifests by chronic cough, exertional dyspnoea, and sputum production. Most patients also have chronic paranasal sinusitis. If left untreated, DPB progresses to bronchiectasis, respiratory failure and death. A significant improvement in the prognosis has been reported thanks to the use of long-term therapy with macrolide antibiotics, the effect of which is attributed to an anti-inflammatory and immunoregulatory action.