TY - JOUR T1 - Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents JF - European Respiratory Journal JO - Eur Respir J SP - 523 LP - 527 DO - 10.1183/09031936.00214713 VL - 44 IS - 2 AU - Caroline Beynat-Mouterde AU - Guillaume Beltramo AU - Guillaume Lezmi AU - Didier Pernet AU - Clio Camus AU - Annlyse Fanton AU - Pascal Foucher AU - Vincent Cottin AU - Philippe Bonniaud Y1 - 2014/08/01 UR - http://erj.ersjournals.com/content/44/2/523.abstract N2 - To the Editor:We identified six patients with clinical, radiographic and physiological features typical of pleuroparenchymal fibroelastosis (PPFE). In the six cases, PPFE may have been causally related to prior alkylating drugs used to treat malignacy, namely cyclophosphamide in five of the cases and carmustine (BCNU (1,3-bis-(2-chloroethyl)-1-nitrosourea)) in one. Based on an extensive review of the literature, we suspect that similar cases may have already been reported in the past 4 decades but have not been recognised either as PPFE or as drug-induced in nature.In 2004, Frankel et al. [1] described a then-new clinicopathologic entity, which they termed “idiopathic PPFE”. The authors identified five patients who had a clinical presentation suggestive of chronic interstitial pneumonitis that did not fit any previously established category of idiopathic interstitial pneumonia. The patients were characterised by significant pleural involvement in the form of pleural thickening, which was more marked in both upper regions. In all five patients, surgical biopsy demonstrated prominent visceral pleural fibrosis with subpleural fibroelastosis and an abrupt transition between the fibrotic area and the near-normal adjoining lung. There was sparing of the underlying lung parenchyma at some depth from the pleural surface, and sparse fibroblast foci were present at the edge of the fibrotic area. Since that seminal report, several cases and small series have been reported that have demonstrated a strikingly similar clinical, radiological and pathological presentation, also characterised by difficult-to-treat pneumothoraces in a sizable fraction of affected patients.In the updated American Thoracic Society/European Respiratory Society classification of idiopathic interstitial pneumonias [2], PPFE is included as a separate but well-defined category of rare entities. Little is known about the aetiology of PPFE and … ER -