A case of diffuse panbronchiolitis, associated with severe pulmonary hypertension, managed with bilateral lung transplantation from a brain-dead donor

Respir Investig. 2015 Sep;53(5):242-6. doi: 10.1016/j.resinv.2015.03.001. Epub 2015 May 27.

Abstract

Diffuse panbronchiolitis (DPB) is a chronic respiratory disease that mainly involves the respiratory bronchioles, and has historically been associated with a very poor prognosis. The development of long-term low dose macrolide therapy in the 1980s has dramatically improved the prognosis of DPB. Nevertheless, some cases are resistant to macrolide therapy, and ultimately develop severe respiratory failure and pulmonary hypertension; in such cases lung transplantation is a viable treatment option. Here we report the case of a 40-year-old patient with a 20-year history of DPB, who underwent bilateral lung transplantation due to severe respiratory failure with pulmonary hypertension.

Keywords: Brain-dead donor; Chronic sinusitis; Diffuse panbronchiolitis; Lung transplantation; Pulmonary hypertension.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Brain Death*
  • Bronchiolitis / complications*
  • Bronchiolitis / surgery*
  • Female
  • Haemophilus Infections / complications*
  • Haemophilus Infections / surgery*
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / surgery*
  • Lung Transplantation / methods*
  • Respiratory Insufficiency / etiology
  • Respiratory Insufficiency / surgery
  • Severity of Illness Index
  • Tissue Donors*
  • Treatment Outcome

Supplementary concepts

  • Diffuse panbronchiolitis