TBLB by cryoprobe is safe and potentially useful in the diagnosis of ILD. Larger multisite randomized trials are required to confirm the potential benefits of this procedure. Clinical trial registration at ClinicalTrials.gov: NCT01064609.

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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.