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Idiopathic inflammation and obstruction of bronchioles, eventually accompanied by bronchiectasis; cases originally reported are almost all from Japan.
Synonym(s): diffuse panbronchiolitis
diffuse panbronchiolitisA 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.
Chronic sinusitis, wheezing, crackles, dyspnoea, purulent sputum, hypoxia, hypercapnia, pulmonary hypertension, cor pulmonale and eventually respiratory failure.
Without erythromycin, 10-year survival is 33%; with it, 90%.