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Insights Into the Preclinical Models of SSc

  • Scleroderma (S Bhattacharyya, Section Editor)
  • Published:
Current Treatment Options in Rheumatology Aims and scope Submit manuscript

Abstract

Purpose of Review

In the recent decade, new animal models which phenocopy a broad range of systemic sclerosis (SSc)-like features have been generated. This review article outlines the strengths and limitations of 4 animal models, which were established or further characterized in the recent few years.

Recent Findings

Inducible SSc animal models, such as hypochlorous acid-treated mice and type V collagen-immunized mice and rabbits, recapitulate multiple organ involvement of SSc, including diffuse skin fibrosis, interstitial lung disease, pulmonary arterial hypertension, and/or kidney involvement. Further characterization of vascular aspects in Psgl1−/− mice shed new light on the endothelium-leukocyte interaction in immune tolerance underlying SSc-like vasculopathy. In vitro studies with dermal microvascular endothelial cells, bone marrow-derived endothelial progenitor cells, and bone marrow-derived mesenchymal stem cells, a precursor of pericytes, derived from Klf5+/−;Fli1+/− mice dissect the aberrant roles of angiogenesis, vasculogenesis, and anastomosis in SSc-like vasculopathy.

Summary

SSc animal models which reproduce a broad range of organ involvement support our understanding of an SSc-specific pathological cascade, but no current animal models mimic the whole range of SSc pathological features. However, a proper combination of complimentary models can help us better understand the molecular pathogenesis of SSc and promote the development of new therapies.

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  1. Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

    Yoshihide Asano

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Asano, Y. Insights Into the Preclinical Models of SSc. Curr Treat Options in Rheum 7, 334–348 (2021). https://doi.org/10.1007/s40674-021-00187-w

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