Histological evolution of pleuroparenchymal fibroelastosis

Takako Hirota, Yuji Yoshida, Yasuhiko Kitasato, Michihiro Yoshimi, Takaomi Koga, Nobuko Tsuruta, Masato Minami, Taishi Harada, Hiroshi Ishii, Masaki Fujita, Kazuki Nabeshima, Nobuhiko Nagata, Kentaro Watanabe

    Research output: Contribution to journalArticlepeer-review

    40 Citations (Scopus)


    Aims: To investigate the histological evolution in the development of pleuroparenchymal fibroelastosis (PPFE). Methods and results: We examined four patients who had undergone surgical lung biopsy twice, or who had undergone surgical lung biopsy and had been autopsied, and in whom the histological diagnosis of the first biopsy was not PPFE, but the diagnosis of the second biopsy or of the autopsy was PPFE. The histological patterns of the first biopsy were cellular and fibrotic interstitial pneumonia, cellular interstitial pneumonia (CIP) with organizing pneumonia, CIP with granulomas and acute lung injury in cases 1, 2, 3, and 4, respectively. Septal elastosis was already present in the non-specific interstitial pneumonia-like histology of case 1, but a few additional years were necessary to reach consolidated subpleural fibroelastosis. In case 3, subpleural fibroelastosis was already present in the first biopsy, but only to a small extent. Twelve years later, it was replaced by a long band of fibroelastosis. The septal inflammation and fibrosis and airspace organization observed in the first biopsies were replaced by less cellular subpleural fibroelastosis within 3-12 years. Conclusions: Interstitial inflammation or acute lung injury may be an initial step in the development of PPFE.

    Original languageEnglish
    Pages (from-to)545-554
    Number of pages10
    Issue number4
    Publication statusPublished - Mar 1 2015

    All Science Journal Classification (ASJC) codes

    • Pathology and Forensic Medicine
    • Histology


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