Pleuroparenchymal fibroelastosis as a series of airway complications associated with chronic graft-versus-host disease following allogeneic bone marrow transplantation

Intern Med. 2014;53(1):43-6. doi: 10.2169/internalmedicine.53.1124.


We herein report the case of a 31-year-old woman who presented with bilateral upper lobe volume loss and pleural irregularities with hilar retraction. She had undergone allogeneic bone marrow transplantation (BMT) for the treatment of acute lymphoblastic leukemia nine years earlier. A surgical lung biopsy showed pleural thickening and subpleural alveolar collapse and fibrosis, consistent with a diagnosis of pleuroparenchymal fibroelastosis (PPFE). Antecedent sicca syndrome and the absence of other causes of fibroelastosis suggested that these abnormalities were associated with chronic graft-versus-host disease (cGVHD). PPFE as a late, noninfectious complication is rare; however, the present case suggests a new class of BMT-related pulmonary complications associated with cGVHD.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Allografts
  • Bone Marrow Transplantation / adverse effects*
  • Chronic Disease
  • Female
  • Graft vs Host Disease / diagnostic imaging*
  • Graft vs Host Disease / etiology*
  • Humans
  • Pulmonary Fibrosis / diagnostic imaging*
  • Pulmonary Fibrosis / etiology*
  • Radiography
  • Transplantation, Homologous / adverse effects