Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents

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

“…The condition does not clearly fit into any of the previously described interstitial pneumonias and, thus, is now specifically included in the classification of idiopathic interstitial pneumonias (IIP) under the heading ''rare IIP'' [2,10]. The six patients in the study by BEYNAT-MOUTERDE et al [1] are reminiscent of the 78 cases (59 in the English literature) of PPFE that have been published since the initial reports from Japanese investigators (to whom credit is due), under the term ''upper lobe fibrosis'' or ''Amitani's disease'' [11][12][13], as well as initial reports in the western world by FRANKEL et al [3]. The three largest series of PPFE included nine [14] 12 [8] and 15 [15] cases, respectively.…”

“…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).…”

“…There was a history (6 months to 16 years previously) of multi-agent chemotherapy in all six patients; the alkylators cyclophosphamide (n55) and BCNU (bis-chloroethylnitrosourea; n51) were the common denominators in their treatment. BEYNAT-MOUTERDE et al [1] raised the possibility that alkylating drugs may have triggered or caused PPFE. Issues raised by their study relate to: 1) PPFE as a clinically, radiographically and pathologically separate and recognisable entity; 2) possible aetiological factors at the origin of PPFE, including drugs; and 3) current management strategies.…”

“…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 (n55) and BCNU (bis-chloroethylnitrosourea; n51) were the common denominators in their treatment.…”

“…Presenting features include chronic dull pleuritic pain with episodes of a sharper quality (which may not reflect pneumothorax in all patients), platythorax [26] (which can be measured on computed tomography (CT) or with a calliper), and small chronic spontaneous uni-or bilateral pneumothoraces [27]. On imaging, the fibrotic pleura and subjacent collapsed and fibrotic lung manifest as a dense irregular peripheral rim [1,8]. Wedge-shaped pleural-based densities protrude along parenchymal septa toward the hila [8,9], and the latter tend to be retracted upwards (figs 1 and 3) [24].…”