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Fibrogenesis, novel lessons from animal models

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Abstract

Systemic sclerosis (SSc) is a devastating chronic autoimmune connective tissue disease characterized by vasculopathy, autoimmunity with inflammation, and progressive fibrogenesis. The current paradigm of the pathogenesis of SSc is that of an unknown initial trigger, leading to a complex interaction of immune cells, endothelial cells, and fibroblasts, producing cytokines, growth and angiogenic factors, and resulting in uncontrolled and persistent tissue fibrogenesis by an altered mesenchymal cell compartment. Animal models are of utmost importance to investigate the different steps in the pathogenesis. This review will elaborate on recent findings in established and more recently developed animal models, presenting data on compounds that are in or ready to be translated into clinical trials, or provide interesting new findings in the understanding of the pathophysiology of SSc. We focus on recent findings concerning the vessel—extracellular matrix interaction, the initial triggering aggressor, the concept of autoimmunity and inflammatory changes, the effector cells and their origins, and the complex interaction of the different signaling pathways in fibrogenesis.

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  1. Laboratory of Tissue Homeostasis and Disease, Skeletal Biology and Engineering Research Center, Department of Development and Regeneration, KU Leuven, Herestraat 49, 3000, Leuven, Belgium

    Ellen De Langhe & Rik Lories

  2. Division of Rheumatology, University Hospitals Leuven, Herestraat 49, 3000, Leuven, Belgium

    Ellen De Langhe & Rik Lories

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  1. Ellen De Langhe
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Correspondence to Rik Lories.

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This article is a contribution to the Special Issue on Immunopathology of Systemic Sclerosis - Guest Editors: Jacob M. van Laar and John Varga

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De Langhe, E., Lories, R. Fibrogenesis, novel lessons from animal models. Semin Immunopathol 37, 565–574 (2015). https://doi.org/10.1007/s00281-015-0510-8

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  • DOI: https://doi.org/10.1007/s00281-015-0510-8

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