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RESEARCH PRODUCT

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

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In this issue of the European Respiratory Journal , Beynat-Mouterde et al. [1] 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. 1). Presentation in all six patients was similar with cough, dyspnoea, occasional chest pain and weight loss. Imaging was distinctive and showed a cephalad, irregular, pleural-based thickening that encroached on the lung bilaterally. Five patients presented with “platythorax” (fig. 2), a preferential reduction in the anterio-posterior diameter of the chest wall. 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 (n=3) and brain tumour (n=1). Figure 1– a) Frontal chest radiograph in a patient following chemotherapy for acute lymphoblastic leukaemia showing a typical appearance of pleuropulmonary fibroelastosis (corresponding to case 2 in [1]). Note the markedly thickened pleural dome on the right and partial spontaneous pneumothorax on the left. b) Computed tomography scan of the same patient taken several months later. A chest tube had been inserted and removed in the interim. A smaller, persistent pneumothorax is visible. Figure 2– Advanced cases may show markedly restrictive physiology and reduced anterio-posterior diameter of the thorax; a trait known as platythorax. Pathology in four patients showed a pattern consistent with pleuropulmonary fibroelastosis (PPFE). Due to similar clinical imaging presentation, two additional patients without biopsy were included in the study of Beynat-Mouterde et al. [1]. There was a history (6 months to 16 years previously) of multi-agent chemotherapy in all six patients; the alkylators cyclophosphamide (n=5) and BCNU (bis-chloroethylnitrosourea; n=1) …