ObjectiveTo enhance the cognition about the clinical characteristics of diffuse panbronchiolitis (DPB). MethodsThe data of patients with DPB searched out on the computer from January 1996 to September 2013 were retrospectively studied. ResultsThe 91 patients had a male to female ratio of 2︰1, and the mean age at onset was (40.5± 18.4) years old. The median course of disease was 7 years. The main clinical profiles included chronic cough, sputum production, exertional dyspnea, and crackles. A history of sinusitis occurred in 90.1% (82/91) of the patients. Positive rate of cold hemoagglutinin and HLA-B54 were 46.1% (30/65) and 42.1% (8/19), respectively. All patients' CT scans showed bilateral, diffuse, small centrilobular nodules. Lung function assessment showed an obstructive ventilation disfunction in 60.0% (51/85) of the patients and a mixed obstructive-restrictive pattern in 36.5% (31/85) of the patients. Thirty-four patients underwent lung biopsy. A total of 65.9% (60/91) of the patients had been misdiagnosed, and 98.8% (84/85) of the patients achieved significant improvement after the macrolide therapy. ConclusionDPB is not rare in China, but tends to be misdiagnosed or underdiagnosed. Macrolides can improve the prognosis of DPB.