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Related Experiment Videos

Agonistic antibodies in systemic sclerosis.

Gianluca Moroncini1, Silvia Svegliati Baroni1, Armando Gabrielli1

  • 1Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, 60126, Italy.

Immunology Letters
|October 17, 2017
PubMed
Summary

Agonistic autoantibodies targeting cell surface receptors may drive Systemic Sclerosis (SSc) pathogenesis. This review examines autoantibodies with demonstrated in vitro and in vivo activity, potentially fulfilling Koch's postulates for disease causation.

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Area of Science:

  • Immunology
  • Rheumatology
  • Pathophysiology

Background:

  • Systemic sclerosis (SSc) involves microangiopathy, fibrosis, and autoantibodies.
  • The pathogenic role of autoimmunity in SSc phenotypes remains debated.
  • While antinuclear antibodies (ANAs) are common in SSc, their direct pathogenic role is unproven.

Purpose of the Study:

  • To review autoantibodies with demonstrated agonistic activity in Systemic Sclerosis (SSc).
  • To explore autoantibodies that may fulfill Koch's postulates for disease causation.
  • To discuss both agonistic and antagonistic autoantibodies relevant to SSc pathogenesis.

Main Methods:

  • Literature review focusing on autoantibodies with in vitro and in vivo evidence of activity.
  • Analysis of autoantibodies targeting cell surface receptors implicated in SSc.
Keywords:
AECAAT1R/ETARM3RMonoclonal autoantibodiesPDGFRSScSclerodermaSystemic sclerosis

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  • Inclusion of antibodies meeting criteria for potential disease causation.

    • Main Results:

    • Identification of agonistic autoantibodies (e.g., anti-endothelial cells, anti-PDGFR, anti-AT1R, anti-ETaR) with demonstrated activity.
    • Evidence suggests these autoantibodies can directly activate pathways contributing to vascular and tissue damage.
    • Antagonistic autoantibodies (anti-M3R) are also considered for their potential role.

    Conclusions:

    • Certain agonistic autoantibodies show potential as direct drivers of SSc pathogenesis.
    • Further research into these specific autoantibodies is warranted to confirm their causal role.
    • Understanding these autoantibodies could lead to novel diagnostic and therapeutic strategies for SSc.