TY - JOUR
T1 - The Exciting Future for Scleroderma
T2 - What Therapeutic Pathways Are on the Horizon?
AU - Distler, Jörg H.W.
AU - Riemekasten, Gabriela
AU - Denton, Christopher P.
N1 - Copyright © 2023 Elsevier Inc. All rights reserved.
PY - 2023/5
Y1 - 2023/5
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85149733754&partnerID=8YFLogxK
UR - https://www.mendeley.com/catalogue/2ef785b7-703d-3288-96a1-fac61657c2d2/
U2 - 10.1016/j.rdc.2023.01.014
DO - 10.1016/j.rdc.2023.01.014
M3 - Scientific review articles
C2 - 37028846
AN - SCOPUS:85149733754
SN - 0889-857X
VL - 49
SP - 445
EP - 462
JO - Rheumatic Disease Clinics of North America
JF - Rheumatic Disease Clinics of North America
IS - 2
ER -