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Podoplanin-positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis

Yasunori Enomoto1,2, Sayomi Matsushima1,2, Shiori Meguro1

  • 1Department of Regenerative and Infectious Pathology, Hamamatsu University School of Medicine, Shizuoka, Japan.

Histopathology
|February 23, 2018
PubMed

Insights

Podoplanin-positive myofibroblasts may serve as a diagnostic marker for distinguishing pleuroparenchymal fibroelastosis (PPFE) from usual interstitial pneumonia (UIP). This finding suggests PPFE shares a common pathological process with pulmonary apical cap (PAC).

Area of Science:

  • Pulmonary pathology
  • Immunohistochemistry
  • Fibroelastosis

Background:

  • Distinguishing pleuroparenchymal fibroelastosis (PPFE) from usual interstitial pneumonia (UIP) and pulmonary apical cap (PAC) is challenging due to overlapping pathological features.
  • Currently, no specific immunostaining markers exist to differentiate these conditions.

Purpose of the Study:

  • To identify specific immunostaining markers for differentiating PPFE from UIP and PAC.
  • To investigate the role of pleural mesothelial cell-related markers in PPFE pathogenesis.

Main Methods:

  • Immunohistochemistry was performed on lung sections from patients with PPFE (n=4), UIP (n=10), and PAC (n=3).
  • Markers assessed included cytokeratin-5/6, CAM5.2, WT-1, calretinin, desmin, and podoplanin.
  • Immunofluorescence was used to verify findings.

Main Results:

  • Podoplanin was positive in spindle cells within thickened pleura and subpleural fibroelastosis lesions in PPFE and PAC lungs.
  • These podoplanin-positive cells also stained for α-smooth muscle actin, indicating myofibroblast presence.
  • UIP lungs lacked these podoplanin-positive myofibroblasts in pleural and subpleural regions.
  • No significant immunohistochemical or morphological differences were observed between PPFE and PAC.

Conclusions:

  • The presence of podoplanin-positive myofibroblasts may be a pathological hallmark distinguishing PPFE from UIP.
  • This finding suggests a distinct pathogenic process in PPFE that is shared with PAC.
  • Further research is needed to elucidate the precise role of these myofibroblasts in PPFE pathogenesis.
Keywords:
myofibroblastpleuroparenchymal fibroelastosispodoplaninpulmonary apical capusual interstitial pneumonia

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