Systemic Sclerosis (SSc)
Systemic Sclerosis is an uncommon autoimmune disease that impacts the skin and internal organs and poses a significant risk of morbidity and mortality. Systemic Sclerosis research can greatly benefit from dedicated drug and therapy development services. Our company can meet all of your drug and therapy development services systemic sclerosis therapy needs.
Introduction to Systemic Sclerosis
As a multi-faceted autoimmune disease systemic sclerosis is accompanied by changes in skin structure, presence of fibrosis in internal organs, vascular disorders, and immune system dysfunction. It is quite uncommon and its worldwide prevalence is highly variable with a mean figure of approximately 1.4 per 100,000 person-years, with women being affected more than men. The presentation of the disease is highly heterogeneous which adds to the complexity in the diagnosis and management of the condition.
Pathogenesis of Systemic Sclerosis
Systemic Sclerosis results from the activation of fibroblasts through an aberrant immune response mediated by cytokines and autoantibodies from immune cells such as macrophages and B cells. This leads to a pathological state of fibrosis with excessive collagen deposition in tissues. Endothelial injury, vasculopathy, and the resultant negative consequences such as Raynaud's phenomenon and pulmonary hypertension add further complexity to the disease.
Fig.1 Key pathways involved in Systemic Sclerosis pathogenesis and therapeutic targets of current and emerging therapies. (Bukiri, H. and Volkmann, 2022)Diagnostics Development of Systemic Sclerosis
- Autoantibody testing:
Antinuclear antibodies (ANA), anti-topoisomerase antibodies (anti-Scl-70), and anti-centromere antibodies can help diagnose.
- Inflammatory markers:
Individuals commonly have elevated serum inflammatory markers such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR).
- Biomarker development:
Research is ongoing to identify biomarkers to diagnose the disease early and predict its progression.
Therapy Development of Systemic Sclerosis
Small dosage chemical agents, such as Nintedanib and Pirfenidone, used for treating Systemic Sclerosis-related interstitial lung disease (ILD) are aimed at the therapeutic of Systemic Sclerosis. These agents block the processes of inflammation and fibrosis via tyrosine kinases and TGF-beta signal transduction respectively.
MSCs (Mesenchymal Stem Cells) and HSCT (Hematopoietic Stem Cell Transplantation) are examples of Cell therapies that attempt to use immune system modulation and tissue regeneration to manage Systemic Sclerosis. While HSCT may provide lasting relief from symptoms, MSCs work to combat fibrosis.
Rituximab and Tocilizumab are examples of Monoclonal antibodies used to tackle the symptoms of Systemic Sclerosis by acting on the immune system. Thus, through inhibition of CD20 and suppression of IL-6, they mitigate muscle weakness and skin fibrosis, which, ultimately, block the advancement of the disease.
Attempts have been made to address Systemic Sclerosis through Gene Therapies by modifying or silencing genes that drive disease progression, such as implementing antisense oligonucleotides against the TGF-beta mRNA. These are mainly clinical trials, but they do present opportunities for therapy in the future.
Our Services
We partner with our clients in designing novel therapies for Systemic Sclerosis. Our group works tirelessly on these custom-designed approaches in order to make sure you achieve your goals while being supported at every step of the process.
Platforms of Systemic Sclerosis Therapy Development
Animal Models of Systemic Sclerosis
We have acquired the capacity to develop and make use of relevant animal models of the disease and its therapy. These models permit the testing of safety and efficacy for the new therapies.
| Non-Genetically Engineering Models | ||
| Our company specializes in delivering top-notch services to create NON-GEMs. We provide diverse model choices customized to meet specific research needs related to Systemic Sclerosis. | ||
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| Genetically Engineered Models | ||
| Our expertise in genetic engineering techniques, technology, allows us to generate accurate and reliable models that recapitulate the genetic alterations observed in human Systemic Sclerosis. | ||
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| Optional Species | Mice, Rats, Non-human primates, Others | |
In addition to these models, our comprehensive services encompass other models that target specific signaling pathways and molecular targets.
If our services resonate with you, we invite you to contact us whenever works best for you. Let's discuss customizing our solutions to align seamlessly with your objectives and aspirations.
References
- Bukiri, H. and Volkmann, E.R., "Current advances in the treatment of systemic sclerosis." Curr Opin Pharmacol, (2022). 64: p. 102211.
- Rosendahl, A.H., et al., "Pathophysiology of systemic sclerosis (scleroderma)." Kaohsiung J Med Sci, (2022). 38(3): p. 187-195.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.