Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

Abstract

A 26-year-old woman presented with a 15-year history of non-progressive dyspnoea. Chest imaging showed bilateral apical pleural and parenchymal scarring, pleural thickening and bronchiectasis. Pulmonary function tests showed a moderate restrictive defect. Non-invasive workup was non-revealing; therefore, the patient was referred for video-assisted thoracic surgery and lung biopsy. Histopathology revealed pleural thickening and, subpleural parenchymal fibrosis and elastic tissue deposition. Lung parenchyma further away from the pleura was well preserved. Based on these findings, the patient was diagnosed with pleuroparenchymal fibroelastosis (PPFE). Since PPFE is a progressive disorder without effective medical therapies, and given our patient’s worsening symptoms, she underwent bilateral lung transplantation. It has been almost 4 years since the lung transplantation, our patient continues to do well. To the best of our knowledge, to date, this is the longest follow-up reported for a PPFE patient undergoing lung transplantation.

Idiopathic pleuroparenchymal fibroelastosis confirmed by pathology: a case report

“…Most pulmonary function damage showed restrictive ventilation dysfunction, some of which were associated with the concomitant complication of diffusion dysfunction, and there were a few cases with normal pulmonary ventilation function or obstructive ventilation dysfunction. 7 The patient whose case is reported here also showed these symptoms. However, further information for a diagnosis was needed including imaging and pathological manifestations because her clinical manifestations were not specific.…”

Lung Transplantation for Pleuroparenchymal Fibroelastosis

“…Righi et al [ 53 ] also described a patient with IPPFE who required long-term rehabilitation after LT. We previously reported a case of fatal secondary pulmonary hypertension associated with flat chest in a patient with IPPFE who underwent single LT [ 54 ]. Other authors have also reported complicated post-LT courses [ 55 , 56 , 57 ]; however, after recovery from early complications, such as chylothorax, dysphagia, vocal cord paralysis, pneumonia, and repeated nausea and vomiting for an unknown reason, the patients apparently did well. There are also several case reports of LT for IPPFE or non-idiopathic PPFE without detailed information about the post-LT course [ 58 , 59 , 60 ].…”

Pleuroparenchymal fibroelastosis

“…Experience with lung transplantation is still limited [13,67–69]. Shiiya et al retrospectively reviewed all 31 patients with idiopathic PPFE and 69 patients with IPF who underwent lung transplantation in Japan over a 20-year period, and found no difference in survival between groups [70].…”