Faculty, Staff and Student Publications
Publication Date
11-1-2023
Journal
Current Opinion in Rheumatology
Abstract
Purpose of review: Systemic sclerosis (SSc) is a multisystem autoimmune connective tissue disease characterized by early inflammation followed by excessive fibrosis in the skin and internal organs. Enhancing our comprehension of SSc pathogenesis is essential to develop effective therapeutic strategies. Animal models that mimic one or more aspects of SSc have been proven to be a valuable resource for investigating disease mechanisms. This review aims to provide an updated overview of the existing SSc animal models and the potentially relevant pathways to SSc pathogenesis.
Recent findings: This review focuses on the most recently generated and investigated animal models, which delve into novel pathways beyond existing models or employ genetic technologies to gain a deeper understanding of SSc pathogenesis including activation of early type I interferon (IFN) signaling pathway, immune cell function and pulmonary artery hypertension (PAH).
Summary: While no single animal model can fully replicate SSc, a combination of different models can offer valuable insights into the pathways involved in the onset and advancement of the SSc. These insights can prove animal models as a crutial preclinical tool for developing effective treatments for SSc.
Keywords
Animals, Humans, Scleroderma, Systemic, Fibrosis, Autoimmune Diseases, Inflammation, Disease Models, Animal, systemic sclerosis, fibrosis, animal model
DOI
10.1097/BOR.0000000000000967
PMID
37605874
PMCID
PMC10553484
PubMedCentral® Posted Date
11-1-2024
PubMedCentral® Full Text Version
Author MSS
Published Open-Access
yes