Objective  To explore the treatment strategies of pleuroparenchymal fibroelastosis (PPFE).

  Methods  A 22-year-old male patient was complicated with PPFE after receiving chemotherapy in combination with stem cell transplantation for lymphoma. He underwent thoracoscopic left lung tongue wedge resection, bilateral pleurodesis followed by allogeneic left lung transplantation. Literature review was performed to analyze the etiology, pathogenesis, imaging features, pathological features and treatment of PPFE.

  Results  The PPFE patient required the non-invasive ventilator for 24 h before lung transplantation. After lung transplantation, the shortness of breath and respiratory failure were cured and the quality of life was significantly improved. No eligible studies was found in the domestic database, and 26 literatures published in English were found in the international databases. Among them, 9 literatures (case reports) were finally included after screening. PPFE could be divided into the primary and secondary categories according to the etiology. The clinical manifestations of PPFE mainly included dry cough, dyspnea on exertion, chest pain, repeated pneumothorax and body weight loss. Chest CT scan demonstrated irregular thickening of the pleura in bilateral upper lungs. Pathological manifestations consisted of evident thickening of the visceral pleura, fibroelastosis and arrangement disorder in the pleura and the underlying pulmonary interstitium. PPFE could progress rapidly. Adrenocortical hormone and other immunosuppressive agents yielded low clinical efficacy and poor clinical prognosis. Lung transplantation was a necessary treatment for PPFE.

  Conclusions  PPFE cannot be effectively treated by conservative therapy. It is recommended to deliver lung transplantation as early as possible.