A severe idiopathic inflammatory condition that diffusely involves the bronchioles which, if untreated, leads to bronchiectasis. Macrolide antibiotics–e.g., erythromycin, slow but don’t halt or reverse disease progression.
Clinical findings Chronic sinusitis, wheezing, crackles, dyspnoea, purulent sputum, hypoxia, hypercapnia, pulmonary hypertension, cor pulmonale and eventually respiratory failure.
Pathology Nodules of foamy macrophages, heterogeneous population of CD4+ and CD8+ T-cells in the lymphocytic infiltrate, virtual absence of B lymphocytes in foci of inflammation, lumina filled with neutrophils, distortion of alveoli and formation of microabscesses
Prognosis Without erythromycin, 10-year survival is 33%; with it, 90%
Reference Diagnostic Pathology 2014, 9:12