Systemic sclerosis (SSc) is a chronic autoimmune disease of unknown cause, which leads to disability and may cause premature death. SSc is characterized by massive accumulation of extracellular matrix proteins (=fibrosis) in skin and internal organs, such as lungs, with permanent loss of organ function.
Metabolic alterations have been recently recognized as an important pathogenic process underlying fibrosis. Decreasing/reversing fibrosis in patients with SSc can improve the prognosis of this devastating disease.
Project LeadB Burja
We aim at exploring the crosstalk between metabolic and fibrotic pathways in SSc fibroblasts to uncover new anti-fibrotic treatment strategies. We will explore dysregulation of metabolic pathways in SSc fibroblasts and determine, whether metabolic substrates, such as alpha-ketoglutarate (αKG) influence the pro-fibrotic activities of skin and lung fibroblasts. Targeting metabolic pathways might reverse fibrotic activation and halt fibrosis in SSc with direct implications for drug discovery in SSc.
Patients with SSc will be involved in the preparation of informed consent forms and communicating our research findings to public. Patients will actively participate in the development of research projects. We will promote the EULAR’s initiative ‘Patients - research partners’. This will establish long-term partnerships between rheumatologists, researchers and patients in Slovenia.