Platelets in Systemic Sclerosis: the Missing Link Connecting Vasculopathy, Autoimmunity, and Fibrosis?

  • Konstantinos Ntelis
  • Dimitrios Bogdanos
  • Theodoros Dimitroulas
  • Lazaros Sakkas
  • Dimitrios DaoussisEmail author
Scleroderma (J Varga, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Scleroderma


Purpose of Review

Platelets are no longer recognized solely as cell fragments regulating hemostasis. They have pleiotropic functions and they are linked directly or indirectly with the three cornerstones of systemic sclerosis (SSc): vasculopathy, autoimmunity, and fibrosis. In this review, we summarize the current knowledge on the potential role of platelets in the pathogenesis of SSc.

Recent Findings

Experimental evidence suggests that vasculopathy, a universal and early finding in SSc, may activate platelets which subsequently release several profibrotic mediators such as serotonin and transforming growth factor β (TGFβ). Platelets may also cross-react with the endothelium leading to the release of molecules, such as thymic stromal lymphopoietin (TSLP), that may trigger fibrosis or sustain vascular damage. Finally, activated platelets express CD40L and provide costimulatory help to B cells, something that may facilitate the breach in immune tolerance.


Preclinical studies point to the direction that platelets are actively involved in SSc pathogenesis. Targeting platelets may be an attractive therapeutic approach in SSc.


Systemic sclerosis Scleroderma Platelets Serotonin TGFβ PDGF VEGF Microparticles 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Konstantinos Ntelis
    • 1
  • Dimitrios Bogdanos
    • 2
  • Theodoros Dimitroulas
    • 3
  • Lazaros Sakkas
    • 2
  • Dimitrios Daoussis
    • 4
    Email author
  1. 1.Department of RheumatologyAgios Andreas HospitalPatrasGreece
  2. 2.Department of Rheumatology, Faculty of Medicine, School of Health SciencesUniversity of ThessalyLarissaGreece
  3. 3.4th Department of Internal Medicine Hippokration Hospital, Medical SchoolAristotle University of ThessalonikiThessalonikiGreece
  4. 4.Department of Internal Medicine, Division of RheumatologyPatras University HospitalPatrasGreece

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