Systemic Sclerosis (SSc)
systemic sclerosis (SSc) is a rare, autoimmune connective tissue disease of unknown origin affecting the skin and visceral organs, which leads to excess morbidity and premature mortality. Specialized drug and therapy development services are essential to enhance and expedite Systemic Sclerosis research. Our company is well-equipped to address your drug and therapy development requirements in Systemic Sclerosis therapy.
Introduction to Systemic Sclerosis
Systemic Sclerosis is a multifaceted autoimmune disease marked by skin and internal organ fibrosis, vascular alterations, and immune system dysregulation. The disease is relatively rare, with global incidence rates varying considerably, but generally found to be around 1.4 per 100,000 person-years. It primarily affects women more than men, and its presentation can vary significantly from one individual to another, contributing to the complexity of its management and diagnosis.
Pathogenesis of Systemic Sclerosis
Systemic Sclerosis is driven by an abnormal immune response that activates fibroblasts through cytokines and autoantibodies from immune cells like macrophages and B cells, leading to excessive collagen production and subsequent tissue fibrosis. Vascular issues, including endothelial damage and vasculopathy, exacerbate complications such as Raynaud's phenomenon and pulmonary hypertension, highlighting the disease's complexity.
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 molecule drugs target molecular pathways to treat Systemic Sclerosis, such as Nintedanib and Pirfenidone for Systemic Sclerosis-related interstitial lung disease (ILD). These drugs inhibit fibrosis and inflammation by targeting tyrosine kinases and TGF-beta respectively.
Cell therapies, including Mesenchymal Stem Cells (MSCs) and Hematopoietic Stem Cell Transplantation (HSCT), aim to treat Systemic Sclerosis through immune modulation and tissue repair. MSCs reduce fibrosis while HSCT may offer long-term symptom remission.
Monoclonal antibodies like Rituximab and Tocilizumab target specific molecules in the immune system to alleviate Systemic Sclerosis symptoms. They help reduce muscle weakness, skin fibrosis, and disease progression by blocking molecules like CD20 and IL-6.
Gene therapies attempt to treat Systemic Sclerosis by modifying or silencing genes linked to the disease progression, such as using antisense oligonucleotides targeting TGF-beta mRNA. These therapies are mostly experimental but show potential for future therapy possibilities.
Our Services
We collaborate with clients to develop innovative therapies for Systemic Sclerosis. Our team dedicates itself to crafting personalized strategies that align with your objectives, ensuring you are supported at every step.
Platforms of Systemic Sclerosis Therapy Development
Animal Models of Systemic Sclerosis
We have established expertise in developing and utilizing relevant animal models that closely mimic the disease characteristics and response to therapy. These models enable us to evaluate the safety and efficacy of potential 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. | ||
| Optional Models |
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| Genetically Engineered Models | ||
| Our expertise in genetic engineering techniques, such as CRISPR/Cas9 technology, allows us to generate accurate and reliable models that recapitulate the genetic alterations observed in human Systemic Sclerosis. | ||
| Optional Models |
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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.