Purinergic signalling in systemic sclerosis

Hoeppner J, Bruni C, Distler O, Robson SC, Burmester GR, Siegert E, Distler J (2021)

Publication Type: Journal article, Review article

Publication year: 2021

Journal

Abstract

SSc is a chronic autoimmune rheumatic disease that involves numerous organs and presents major management challenges. The histopathologic hallmarks of SSc include vasculopathy, fibrosis and autoimmune phenomena involving both innate and adaptive immune systems. Purinergic signalling is a pathway that may be implicated in the pathophysiology of several of these disease manifestations. Extracellular purines are potent signalling mediators, which have been shown to be dysregulated in SSc. As examples, purines can exacerbate vasculopathy and provoke platelet dysfunction; as well as contributing to immune dysregulation. Elements of purinergic signalling further promote organ and tissue fibrosis in several disease models. Here, we provide an overview of extracellular purine metabolism in purinergic signalling and link disorders of these to the molecular pathology of SSc. We also discuss targeting the purinergic signalling and explore the translational applications for new therapeutic options in SSc.

Authors with CRIS profile

Jörg Distler Professur für Molekulare Mechanismen der Organfibrose

Involved external institutions

Charité - Universitätsmedizin Berlin

Germany (DE) Università degli Studi di Firenze / University of Florence

Italy (IT) University of Zurich / Universität Zürich (UZH)

Switzerland (CH) Harvard University

United States (USA) (US)

How to cite

APA:

Hoeppner, J., Bruni, C., Distler, O., Robson, S.C., Burmester, G.R., Siegert, E., & Distler, J. (2021). Purinergic signalling in systemic sclerosis. Rheumatology. https://doi.org/10.1093/rheumatology/keab859

MLA:

Hoeppner, Jakob, et al. "Purinergic signalling in systemic sclerosis." Rheumatology (2021).

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