Systemic sclerosis & Raynaud's
Endothelial Response to Type I Interferon Contributes to Vasculopathy and Fibrosis and Predicts Disease Progression of Systemic Sclerosis
Authors' conclusion
The EC IFN-1 signature distinguished SSc skin subtypes and disease progression and may contribute to vasculopathy and fibrosis.
Study context
- Population
- Skin biopsies from patients with systemic sclerosis (SSc) and controls; discovery (4 SSc, 2 controls), validation data sets GSE138,669 and GSE195452, and an SSc cohortSSc cohort n = 61
- Comparison
- SSc skin vs controls
Methodological detailssingle-cell RNA sequencing, public data set validation, bleomycin plus IFNα-2 adenovirus-associated virus (AAV)-induced model, clinical cohort verification
- Single-cell RNA sequencing
- Public data set validation
- Bleomycin plus IFNα-2 AAV-induced model
- Clinical cohort verification
Main finding
As reported in the abstract
- Endothelial cells (ECs) had the most prominent IFN-1 signature among dermal nonimmune cells
- EC IFN-1 signature increased in SSc vs controls and in dcSSc vs lcSSc
- Capillary EC IFN-1 signature statistically higher in dcSSc > lcSSc > healthy controls (HCs); Endothelial-to-mesenchymal transition (EndoMT) scores increased in parallel
- In the bleomycin plus IFNα-2 AAV model: deteriorated dermal fibrosis, EndoMT and perivascular fibrosis, and blood vessel loss with EC apoptosis
- Vascular MX1 significantly increased in total SSc vs HC skin and in dcSSc vs lcSSc skin
- Baseline vascular MX1 performed similarly to skin score in predicting disease progression over 6 to 34 months in total SSc and was superior in dcSSc
- Discovery and validation data sets showed similar findings
Original publication
Endothelial Response to Type I Interferon Contributes to Vasculopathy and Fibrosis and Predicts Disease Progression of Systemic Sclerosis. Arthritis Rheumatol. Published 27 December 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →