What is it about?

The prognosis of elderly individuals with idiopathic pulmonary fibrosis (IPF) remains poor with a 5-year survival rate worse than several types of cancer. Fibroblasts are central to the progression of pulmonary fibrosis. The pathological hallmark lesions in IPF comprise fibroblastic foci in fibrotic lesions in which aggregates of proliferating fibroblasts and myofibroblasts are involved. Their diversity in fibroblasts makes it difficult to understand the pathogenesis of pulmonary fibrosis. Our study was aimed to determine the role of fibroblasts positive for meflin, identified as a potential marker for mesenchymal stromal cells, during the development of pulmonary fibrosis.

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Why is it important?

Our data show that; 1) meflin expression appeared to be abundant and almost restricted in pulmonary fibroblasts, 2) meflin-positive fibroblasts were localized in fibroblastic foci, not in dense fibrosis, in IPF lungs, 3) pulmonary fibroblasts positive for meflin had anti-fibrotic property to prevent pulmonary fibrosis.


Our study provides the basis to identify the underlying mechanisms by which meflin-expressing fibroblasts emerge in fibrotic foci, not dense fibrosis, of IPF lungs. A new therapeutic strategy for pulmonary fibrosis might be warranted according to our data showing repressive effect of meflin reconstitution into fibroblasts against transforming growth factor-β-induced fibrogenesis. The biological assessment of disease activity in IPF might be also warranted according to our data showing that soluble meflin could be detected in bronchoalvaolar lavage supernatants in parallel with fibrotic activity of bleomycin-induced lung fibrosis.

Naozumi Hashimoto
Nagoya University

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This page is a summary of: Fibroblasts positive for meflin have anti-fibrotic properties in pulmonary fibrosis, European Respiratory Journal, May 2021, European Respiratory Society (ERS),
DOI: 10.1183/13993003.03397-2020.
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