Pleuroparenchymal fibroelastosis: one more walk on the wild side of drugs?

Histology of alkylating chemotherapy in PPFE patients; drugs may cause this rare pattern of lung disease http://ow.ly/yEuOz In this issue of the European Respiratory Journal, report on six young adults (three of whom were female) who developed a clinical imaging pattern of predominant upper lobe fibrosis with apical pneumothoraces (fig. Presentation in all six patients was similar with cough, dyspnoea, occasional chest pain and weight loss. Imaging was distinctive and showed a cephalad, irregular, pleuralbased thickening that encroached on the lung bilaterally. Five patients presented with ''platythorax'' (fig. In all patients, severe restrictive or restrictive-obstructive lung dysfunction progressed to hypoxaemic and hypercarbic respiratory failure, which was fatal in four patients despite intensive care unit support. This outcome was all the more tragic as these four patients had survived previous malignant conditions, including haematological malignancy (n53) and brain tumour (n51).