Idiopathic Pleuroparenchymal Fibroelastosis

Abstract

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.

Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study

“…Compared with other ILDs, the radiologic features of PPFE are very distinct, which has already evoked discussion regarding the possibility of nonpathologic diagnosis [9,10]. However, based on radiologic assessment at a single time point, there would be a risk to misdiagnose pulmonary apical cap as PPFE.…”

“…a Organizing pneumonia with focal UIP-like fibrosis (n = 1); patchy and mild fibrotic alveolitis (n = 1). low body mass index, hypercapnia, high RV/TLC, and high complication rates of pneumothorax [2][3][4][5][6][7][8][9][10][11]. Although validation studies are necessary, these results suggest the applicability of our modified criteria.…”

“…First, is there a need for pathologic evaluation? As several researchers have pointed out, there does not appear to be any effective treatment once the diagnosis is made; patients with idiopathic PPFE frequently show deteriorated pulmonary function and poor prognosis [2,[6][7][8][9][10][11]. Additionally, the risks of postoperative pneumothorax and acute exacerbation of combined interstitial lung disease (ILD), mainly usual interstitial pneumonia (UIP), are serious concerns.…”

“…Additionally, the risks of postoperative pneumothorax and acute exacerbation of combined interstitial lung disease (ILD), mainly usual interstitial pneumonia (UIP), are serious concerns. In this context, a nonpathologic diagnosis of idiopathic PPFE would be appealing when the patient characteristics are clinically and radiologically consistent with the disease [9,10]. Second, Reddy's criteria do not include disease progression.…”

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

“…They used it to describe an upper lobe predominant DPLD in five patients, that was characterised by marked pleural and parenchymal fibroelastosis and for which the pathology findings did not match any of the known DPLDs. Since then there have been around a hundred such cases reported in the literature and it has also been included as a rare DPLD in the American Thoracic Society/European Respiratory Society consensus statement on the multidisciplinary diagnosis of idiopathic interstitial pneumonias 2 3. Interestingly, disease processes very similar to PPFE had also previously been reported as idiopathic pulmonary upper lobe fibrosis and Aminati’s disease 4–6…”