diffuse panbronchiolitis


pan·bron·chi·o·li·tis

(pan'bron-kē-ō-lī'tis), Idiopathic inflammation and obstruction of bronchioles, eventually accompanied by bronchiectasis; cases originally reported are almost all from Japan. Synonym(s): diffuse panbronchiolitis

diffuse panbronchiolitis

A severe idiopathic inflammatory condition that diffusely involves the bronchioles. It is most common in eastern Asian males and, if untreated, leads to bronchiectasis. Macrolide antibiotics—e.g., erythromycin—slows but does not halt or reverse disease progression.
 
Clinical findings
Chronic sinusitis, wheezing, crackles, dyspnoea, purulent sputum, hypoxia, hypercapnia, pulmonary hypertension, cor pulmonale and eventually respiratory failure.
 
Prognosis
Without erythromycin, 10-year survival is 33%; with it, 90%.