The Role of Epithelial Cells in Scleroderma—Second Edition
A special issue of Cells (ISSN 2073-4409).
Deadline for manuscript submissions: 31 January 2025 | Viewed by 103
Special Issue Editor
Interests: inflammatory fibrosis; epithelial cells; scleroderma
Special Issues, Collections and Topics in MDPI journals
Special Issue Information
Dear Colleagues,
This is the expanded second edition of ‘The Role of Epithelial Cells in Scleroderma’, the first edition of which led to the publication of six papers.
Systemic sclerosis (SSc, scleroderma) is a severe connective tissue disease initiated by environmental factors as well as by dysregulated immunity. The epithelial layers of the skin and affected internal organs may play an important role in the pathogenesis, since they are the first site of exposure to factors from the environment and are also highly endowed with antigen-presenting cells and effector T cell populations. Moreover, activated epithelial cells, such as those overlying wounds, are known to actively promote underlying fibroblasts, leading to tissue repair. Intriguingly, we and others have found the scleroderma epidermis to have an activation state resembling the keratinocyte layer overlying wounds and have shown in co-culture experiments that this epidermis promotes fibroblast activation, leading to a pro-fibrotic response. Whilst a full epithelial-to-mesenchyme transition (EMT) was not seen, a partially evoked form can be detected in which keratinocytes are losing epithelial markers and gaining certain fibroblast proteins. These results are aligned with other forms of fibrosis, where epithelial cell–fibroblast crosstalk has been shown to have a role, but a full EMT has not been seen. The importance of these mechanisms in promoting the skin changes in scleroderma and the more general involvement of epithelial cells in promoting lung and gut manifestations require further investigation. The application of specific therapies targeting the epithelial–fibroblast crosstalk in clinical trials might give the most valuable insight overall.
Dr. Richard Stratton
Guest Editor
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Keywords
- fibrosis
- epithelial–fibroblast interaction
- EMT
- cytokeratin 6 and 16
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Related Special Issue
- The Role of Epithelial Cells in Scleroderma in Cells (6 articles)
