Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection?

To the Editor: Idiopathic pleuroparenchymal fibroelastosis is a peculiar pulmonary fibrosis proposed by Frankel et al . [1] in 2003 and is almost the same concept as idiopathic pulmonary upper lobe fibrosis proposed by Amitani et al. [2]. There are no known causes for fibrosis in idiopathic pleuroparenchymal fibroelastosis. Sometimes, pleuroparenchymal fibroelastosis (PPFE) has underlying diseases or conditions, such as collagen vascular diseases, anti-cancer chemotherapy, irradiation, asbestos exposure and bone-marrow transplantation [3]. Herein, we report the case of a female who received living-donor lung transplantation and died of pulmonary fibrosis, which was pathologically compatible with PPFE in addition to constrictive bronchiolitis, which is a manifestation of chronic lung allograft dysfunction (CLAD) [4]. A 30-yr-old female suffering from idiopathic pulmonary arterial hypertension underwent living-donor lung transplantation surgery and received a right lower lobe from her younger sister and a left lower lobe from her mother in December 2003. 20 months after the lung transplantation she had dyspnoea and a chest radiograph disclosed bilateral ground-glass shadows. 1 month later, right open lung biopsy was performed and a diagnosis of interstitial pneumonia was obtained. Pulse therapy with methylprednisolone slightly improved her condition and prednisolone was administered after the pulse therapy. However, bilateral interstitial opacities gradually deteriorated (fig. 1) with increased dyspnoea. 49 months after the lung transplantation, her daily life had worsened to almost whole-day bed rest. 18 days prior to her death she …