Diffuse panbronchiolitis (DPB) is a rare progressive and eventually fatal pulmonary disease first identified in Japan and initially seen predominantly in Southeast Asia. Macrolide antibiotics rapidly reverse symptoms and pathology, and their use increased the 5 and 10-year survival from 50 and 30 percent, respectively, to over 90%. Review of 181 case reports from previous publications found patients with DPB commonly had their pulmonary symptoms preceded by rhinosinusitis, frequently by many years. Long delays in diagnosis for many years were common. The review further identified DPB in all ethnic groups and multiple areas outside of Southeast Asia. Although diagnosis was most commonly made in adults, 13% of the diagnoses were made in children and nine of the adult cases described onset in childhood. Few cases of relapse were reported, but extended periods of monitoring after treatment were not generally present.
Keywords: Interstitial Lung Disease (ILD); diffuse lung disease; diffuse panbronchiolitis; international health; lung pathology.
© 2019 Wiley Periodicals, Inc.