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ABSTRACT Systemic sclerosis (SSc) is an autoimmune fibrotic disease of unknown aetiology that is characterized by vascular changes in the skin and visceral organs. Autologous haematopoietic

stem cell transplantation can improve skin and organ fibrosis in patients with progressive disease and a high risk of organ failure, indicating that cells originating in the bone marrow are

important contributors to the pathogenesis of SSc. Animal studies also indicate a pivotal function of myeloid cells in the development of fibrosis leading to changes in the tissue

architecture and dysfunction in multiple organs such as the heart, lungs, liver and kidney. In this Review, we summarize current knowledge about the function of myeloid cells in fibrogenesis

that occurs in patients with SSc. Targeted therapies currently in clinical studies for SSc might affect myeloid cell-related pathways. Therefore, myeloid cells might be used as cellular

biomarkers of disease through the application of high-dimensional techniques such as mass cytometry and single-cell RNA sequencing. KEY POINTS * Chronic tissue injury, inflammation and

prolonged fibroblast activation lead to fibrosis and organ dysfunction in systemic sclerosis (SSc). * Myeloid cells are crucial antigen-presenting cells, regulators of inflammatory responses

and producers of cytokines, processes that are implicated in fibrogenesis. * Despite comprehensive investigation of myeloid cells, the function of these cells in SSc is not fully

understood. * High-dimensional analysis of myeloid cells might identify a biomarker of SSc onset and progression. * Strategies that target myeloid cell-related pathways might prevent or

reverse tissue fibrosis. Access through your institution Buy or subscribe This is a preview of subscription content, access via your institution ACCESS OPTIONS Access through your

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SIMILAR CONTENT BEING VIEWED BY OTHERS IMMUNE CELL DYSREGULATION AS A MEDIATOR OF FIBROSIS IN SYSTEMIC SCLEROSIS Article 09 November 2022 AN INTERNATIONAL PERSPECTIVE ON THE FUTURE OF

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Immunol._ 198, 4470–4480 (2017). Article  CAS  PubMed  Google Scholar  Download references AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Rheumatology, Center of Experimental

Rheumatology, University Hospital Zurich, Zurich, Switzerland Gabriela Kania, Michal Rudnik & Oliver Distler Authors * Gabriela Kania View author publications You can also search for

this author inPubMed Google Scholar * Michal Rudnik View author publications You can also search for this author inPubMed Google Scholar * Oliver Distler View author publications You can

also search for this author inPubMed Google Scholar CONTRIBUTIONS The authors contributed equally to all aspects of the article. CORRESPONDING AUTHOR Correspondence to Oliver Distler. ETHICS

DECLARATIONS COMPETING INTERESTS The authors declare no competing interests. ADDITIONAL INFORMATION PUBLISHER’S NOTE Springer Nature remains neutral with regard to jurisdictional claims in

published maps and institutional affiliations. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Kania, G., Rudnik, M. & Distler, O. Involvement of the

myeloid cell compartment in fibrogenesis and systemic sclerosis. _Nat Rev Rheumatol_ 15, 288–302 (2019). https://doi.org/10.1038/s41584-019-0212-z Download citation * Published: 05 April

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