Diffuse panbronchiolitis (DPB) is a chronic inflammatory airway disease which was lethal in the past despite combined treatment with
antibiotics and the use of supportive
therapies such as
oxygen administration. Diagnosis of DPB is performed according to the diagnostic criteria that include major items: symptoms, existence of parasinusitis, chest radiographic findings, minor items: physical signs (coarse
crackles), pulmonary functions and blood gas tests, and elevated titers of cold
hemagglutinin. Pathological feature shows thickness of the wall of respiratory bronchiole with infiltration of lymphocytes, plasma cells, and foamy histiocytes expanded into the peribronchiolar area. Many of these
bronchiolitis progress
respiratory failure with
bronchiectasis in advanced stage. Recently, DPB was interested in
genetic predisposition because of high accumulation in the East Asia with the same HLA haplotype. Low-dose of
erythromycin (400-600 mg/day) is a authorized
therapy to improve the survival time of patients with DPB. 14-membered ring
macrolides other than
erythromycin also improve the survival of DPB patients.