The Exciting Future for Scleroderma: What Therapeutic Pathways Are on the Horizon?

Jörg H.W. Distler, Gabriela Riemekasten, Christopher P. Denton*

*Corresponding author for this work

Abstract

Emerging evidence shows that a complex interplay between cells and mediators and extracellular matrix factors may underlie the development and persistence of fibrosis in systemic sclerosis. Similar processes may determine vasculopathy. This article reviews recent progress in understanding how fibrosis becomes profibrotic and how the immune system, vascular, and mesenchymal compartment affect disease development. Early phase trials are informing about pathogenic mechanisms in vivo and reverse translation for observational and randomized trials is allowing hypotheses to be developed and tested. In addition to repurposing already available drugs, these studies are paving the way for the next generation of targeted therapeutics.

Original languageEnglish
JournalRheumatic Disease Clinics of North America
Volume49
Issue number2
Pages (from-to)445-462
Number of pages18
ISSN0889-857X
DOIs
Publication statusPublished - 05.2023

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)

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