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Background   Factors determining the prognosis of diffuse panbronchiolitis (DPB) remain unclear at present. The objective of this study was to identify the prognostic value of concomitant bronchiectasis in the macrolide treatment efficacy and exacerbation risk in DPB patients.Methods   Data of patients initially diagnosed with DPB at the Shanghai Pulmonary Hospital between January 2007 and December 2017 were retrospectively collected and analyzed. The patients were divided into two groups according to the existence of bronchiectasis. Clinical manifestations, laboratory findings, microbiological culture results, as well as exacerbation risks and treatment outcomes, were compared between these two groups. The survival curve and Cox regression analysis models were additionally constructed to further demonstrate the predicting role of bronchiectasis in DPB exacerbation.Results   Baseline data revealed more respiratory symptoms, lower body mass index (BMI), and forced expiratory volume in one second (FEV 1 ) as well as increased isolates of  Pseudomonas aeruginosa  ( P. aeruginosa ) in DPB subjects with bronchiectasis than those without. Furthermore, bronchiectasis was associated with a lower rate of responsiveness to macrolides and increased exacerbation frequency during follow-up. The survival curve and Cox regression analysis showed that comorbid bronchiectasis was linked to increased time to episode relapse, which remained significant even after controlling for BMI, FEV 1 , and  P. aeruginosa  culture results.Conclusion   The coexistence of bronchiectasis predicted a poor outcome of maintenance macrolide therapy and an increased exacerbation risk in DPB subjects, possibly through its impacts on nutritional status, pulmonary function, and  P. aeruginosa  infections.

Prognostic Value of Concomitant Bronchiectasis in Newly Diagnosed Diffuse Panbronchiolitis Patients on a Maintenance Therapy with Macrolides