“…We strongly suspect that our patient′s earlier chemotherapy with cyclophosphamide may have contributed to the development of NSIP‐like change and PPFE, in view of a number of reported cases of PPFE that were associated with cyclophosphamide treatment The case report by Hamada et al . is interesting because the condition was a cyclophosphamide‐induced late‐onset type of disease and the histology of the autopsied lung resembles that of our patient.…”
Pleuroparenchymal fibroelastosis (PPFE) is characterized by upper lobe‐predominant subpleural fibroelastosis. Despite its characteristic uneven distribution, detailed whole‐lung pathological features of PPFE have rarely been studied. We investigated PPFE in the explanted lungs from a 19‐year‐old male patient with a history of chemotherapy. Grossly, the explanted lungs showed upper lobe‐predominant shrinkage with subpleural and central consolidation. Histologically, fibroelastosis was prominent in the perilobular areas and along the bronchovascular bundles. The other areas of the lung showed diffuse, non‐specific interstitial pneumonia (NSIP)–like change with a characteristic increase of septal elastic fibers. In the digital image analysis, the ratio of elastic fibers to whole fibrosis (EF score) was lower in the subpleural areas than in the NSIP‐like lesions, but the EF scores of the latter showed no significant difference between upper and middle/lower lobes. In the present case, the diffusely distributed elastic fiber‐rich NSIP‐like change, probably caused by the earlier chemotherapy, may have been conducive to the development of PPFE. This suggests that some unknown vulnerability of the upper lobe may exist, various primary lesions converging to the upper lobe predominance of PPFE.
“…We strongly suspect that our patient′s earlier chemotherapy with cyclophosphamide may have contributed to the development of NSIP‐like change and PPFE, in view of a number of reported cases of PPFE that were associated with cyclophosphamide treatment The case report by Hamada et al . is interesting because the condition was a cyclophosphamide‐induced late‐onset type of disease and the histology of the autopsied lung resembles that of our patient.…”
Pleuroparenchymal fibroelastosis (PPFE) is characterized by upper lobe‐predominant subpleural fibroelastosis. Despite its characteristic uneven distribution, detailed whole‐lung pathological features of PPFE have rarely been studied. We investigated PPFE in the explanted lungs from a 19‐year‐old male patient with a history of chemotherapy. Grossly, the explanted lungs showed upper lobe‐predominant shrinkage with subpleural and central consolidation. Histologically, fibroelastosis was prominent in the perilobular areas and along the bronchovascular bundles. The other areas of the lung showed diffuse, non‐specific interstitial pneumonia (NSIP)–like change with a characteristic increase of septal elastic fibers. In the digital image analysis, the ratio of elastic fibers to whole fibrosis (EF score) was lower in the subpleural areas than in the NSIP‐like lesions, but the EF scores of the latter showed no significant difference between upper and middle/lower lobes. In the present case, the diffusely distributed elastic fiber‐rich NSIP‐like change, probably caused by the earlier chemotherapy, may have been conducive to the development of PPFE. This suggests that some unknown vulnerability of the upper lobe may exist, various primary lesions converging to the upper lobe predominance of PPFE.
“…It is worth underlining, however, that these two patterns are not mutually exclusive, as recent studies have revealed that their coexistence may be more frequent than previously thought [9•, 20]. Elements of diffuse alveolar damage, alveolar hemorrhage, and obliterative bronchiolitis can be observed, although this seems to occur in transplant patients, rather than in IPPFE [24]. Fig.…”
Section: The Clinical Spectrummentioning
confidence: 99%
“…Although the pooled proportion of post-procedural pneumothorax onset from meta-analyses of literature is not negligible (up to 12%) [25, 26] and its frequency may well be higher in the context of PPFE, the risk-effectiveness profile may be reasonable in selected, healthier IPPFE patients. However, as the clinical and radiological features in these patients are often highly suggestive, the need for a morphological assessment has been debated [24] and a label of “consistent with PPFE” for cases without biopsy has been proposed. At any rate, it is unquestionable that a multidisciplinary approach is mandatory for a conclusive diagnosis of IPPFE.…”
Purpose of the reviewIdiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP). We aim to summarize the current evidence on IPPFE, in terms of clinical features and potential treatments.Recent findingsOverall, there is increasing awareness of PPFE in association with a separate ILD pattern. Although an agreed consensus on diagnosis has yet to be defined, a list of radiological and histopathological criteria has been proposed. Due to the unfavorable risk-benefit profile of surgical lung biopsy in a significant proportion of patients, a potential role for transbronchial lung cryobiopsy has been suggested. At present, lung transplantation remains the only curative option.SummaryThe increasing awareness of this condition among specialists has led to more frequent identification of IPPFE. Large international studies are needed to better characterize pathogenesis and pheno/endotypes of disease, a key step towards the development of effective treatments.
“…The three IPPFE cases among these four cases did not have radiotherapy, chemotherapy or inhalational injuries, which are known aetiologies of intra-alveolar fibrosis with septal elastosis. Some reports have included PPFE cases without pleural thickening [4,5,8,12,13]. Further studies are required to clarify the significance of pleural thickening in the diagnosis of PPFE.…”
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