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Cells
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The Role of Epithelial Cells in Scleroderma—Second Edition
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The Role of Epithelial Cells in Scleroderma—Second Edition

A special issue of Cells (ISSN 2073-4409).

Deadline for manuscript submissions: 30 September 2025 | Viewed by 891

Special Issue Editor

Dr. Richard Stratton

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Guest Editor
Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, UCL Division of Medicine, London NW3 2QG, UK
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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Published Papers (1 paper)

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Review

11 pages, 647 KiB  
Review
Understanding the Role of Epithelial Cells in the Pathogenesis of Systemic Sclerosis
by Lydia Nagib, Anshul Sheel Kumar and Richard Stratton
Cells 2025, 14(13), 962; https://doi.org/10.3390/cells14130962 - 24 Jun 2025
Viewed by 551
Abstract
Systemic sclerosis (SSc) is an autoimmune fibrotic disorder affecting the skin and internal organs, categorized as either limited cutaneous SSc, where distal areas of skin are involved, or diffuse cutaneous SSc, where more extensive proximal skin involvement is seen. Vascular remodelling and internal [...] Read more.
Systemic sclerosis (SSc) is an autoimmune fibrotic disorder affecting the skin and internal organs, categorized as either limited cutaneous SSc, where distal areas of skin are involved, or diffuse cutaneous SSc, where more extensive proximal skin involvement is seen. Vascular remodelling and internal organ involvement are frequent complications in both subsets. Multiple pathogenic mechanisms have been demonstrated, including production of disease-specific autoantibodies, endothelial cell damage at an early stage, infiltration of involved tissues by immune cells, as well as environmental factors triggering the onset such as solvents and viruses. Although not strongly familial, susceptibility to SSc is associated with multiple single nucleotide polymorphisms in immunoregulatory genes relevant to antigen presentation, T cell signalling and adaptive immunity, as well as innate immunity. In addition, several lines of evidence demonstrate abnormalities within the epithelial cell layer in SSc. Macroscopically, the SSc epidermis is pigmented, thickened and stiff and strongly promotes myofibroblasts in co-culture. Moreover, multiple activating factors and pathways have been implicated in the disease epidermis, including wound healing responses, induction of damage associated molecular patterns (DAMPS) and the release of pro-fibrotic growth factors and cytokines. Similar to SSc, data from studies of cutaneous wound healing indicate a major role for epidermal keratinocytes in regulating local fibroblast responses during repair of the wound defect. Since the epithelium is strongly exposed to environmental factors and richly populated with protective immune cells, it is possible that disease-initiating mechanisms in SSc involve dysregulated immunity and tissue repair within this cell layer. Treatments designed to restore epithelial homeostasis or else disrupt epithelial–fibroblast cross-talk could be of benefit in this severe and resistant disease. Accordingly, single cell analysis has confirmed an active signature in SSc keratinocytes, which was partially reversed following a period of JAK inhibitor therapy. Full article
(This article belongs to the Special Issue The Role of Epithelial Cells in Scleroderma—Second Edition)
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