Autoimmune Disease Series--

Systemic Sclerosis

Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by vascular abnormalities, immune activation, and progressive multi-organ fibrosis. Its core pathological mechanisms include autoantibody-mediated B-cell dysregulation, endothelial injury-induced vasculopathy, and fibroblast activation leading to excessive collagen deposition, ultimately causing fibrosis in organs such as the skin, lungs, and heart. Clinically, it is classified into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc), with the latter progressing rapidly, having a poor prognosis, and frequently complicated by interstitial lung disease (ILD) and myocardial fibrosis, resulting in high mortality.

Current Therapeutic Landscape and Research Focus

Current SSc management relies on immunosuppressants and antifibrotic agents, but efficacy remains limited without reversal of fibrosis. Recent research focuses on targeting pathogenic B cells, inflammatory pathways, and fibrosis reversal:

● Dual-target CAR-NK cell therapy (QN-139b): A groundbreaking therapy pioneered by Xu Huiji's team for dcSSc. It targets CD19/BCMA dual antigens, utilizing iPSC-derived CAR-NK cells to clear pathogenic B cells and plasma cells, thereby inducing immune reconstitution.

● JAK inhibitor (Baricitinib): Targets the JAK1/2 pathway to inhibit inflammatory cytokine signaling. Mechanistic studies indicate it downregulates the profibrotic factor IL-1RL1 (ST2) and upregulates the regeneration-associated protein SYT17.

Systemic Sclerosis Drug Targets Related Products

● Target Proteins

CLEC4C
CSB-MP855470HUh7

Measured by its binding ability in a functional ELISA. Immobilized Human CLEC4C at 2μg/mL can bind Anti-CLEC4C recombinant antibody (CSB-RA855470MA1HU)，the EC₅₀ is 7.658-12.99 ng/mL.

IL13
CSB-MP6408HU

The ED₅₀ as determined by the dose-dependent stimulation of the proliferation of TF-1 cells is 2.045-6.215 ng/mL.

IL1RAP
CSB-MP878844HU

Measured by its binding ability in a functional ELISA. Immobilized Human IL1RAP at 2 μg/ml can bind Anti-IL1RAP recombinant antibody (CSB-RA878844MA1HU). The EC₅₀ is 0.9667-1.477 ng/mL.

TNFSF13B
CSB-MP897523HU1

Measured by its binding ability in a functional ELISA. Immobilized TNFSF13B at 10 μg/ml can bind human BCMA (CSB-MP023974HU1), the EC₅₀ of human TNFSF13B protein is 221.3-298.6 ng/ml.