Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD)
Systemic sclerosis-associated interstitial lung disease (SSc-ILD) poses significant challenges due to its intricate nature involving inflammation, fibrosis, and overproduction of collagen which can severely affect the respiratory health of individuals. Our company specializes in rare diseases, which places us in a unique position to provide innovative cutting-edge platforms and solutions that would aid in your research surrounding the SSc-ILD pathogenesis and novel therapy development.
Introduction to SSc-ILD
SSc-ILD is one of the forms of autoimmune diseases that is marked by continuous inflammation of the lungs and development of fibrosis. Each year, roughly 20 cases of SSc per million adults living in the United States get diagnosed with roughly 90% of cases showing association with interstitial lung disease. SSc-ILD is a form of ILD which requires both timely and effective treatment in order to avert lung damage as well as elevate the quality of life and survival rates of the affected individual.
Pathogenesis of SSc-ILD
SSc-ILD has an integrated mechanism, characterized by an altered immune response, activated fibroblasts, and defective healing of wounds. These elements lead to the abnormal collagen and extracellular matrix proteins deposition in lungs, progressive interstitial fibrosis, lung malfunctioning, and ultimately, the respiratory failure (Fig.1)
Fig.1 The pathogenesis of SSc-ILD involves vascular, immunological, and fibrotic processes. (Mirsaeidi, M., et al., 2019)Biomarkers for SSc-ILD Diagnostics
Extremely precise diagnosis and targeted treatment plans are absolutely vital in tackling the ailment SSc-ILD. As a means to assist in the most problematic cases, we suggest the implementation of certain biomarkers that can help in the diagnosis of SSc-ILD patients that have a high probability of disease progression.
- Krebs von den Lungen-6 (KL-6)
- Surfactant proteins A and D (SP-A and SP-D)
- Chemokine ligand 18 (CCL18)
- C-reaction protein (CRP)
- Connective tissue growth factor (CTGF)
- Soluble intercellular adhesion molecule 1 (sICAM-1)
Therapeutics of SSc-ILD
Small Molecule Drugs Therapy
While dealing with SSc-ILD patients systeomatic immune suppression using Cyclophosphamide, Mycophenolate Mofetil, Pomalidomide, Antifibrotics such as Nintedanib, and Pirfenidone have been proven to improve patient’s quality of life and chronic stable SSc-ILD symptoms and disease progression.
Cell Therapy
Severe SSc-ILD is where autologous stem cell transplantation (AHSCT) cell therapy is done comes into play. The premise of this stem cell therapy is that it uses the body’s own stem cells to develop and cure the damaged lung tissues.
Monoclonal Antibodies Therapy
Monoclonal antibodies especially the ones that inhibit the effector pathways of the immune response to SSc-ILD are more in use recently. Examples of monoclonal antibodies that unselectively destroy the CD20 positive B lymphocytes and the human IL-6 receptor alpha chain are Retuximab and TociIizumab, which have also been used to treat SSc-ILD individuals.
Gene Therapy
The so-called disease’s altered immune reactions and fibrotic processes can be corrected using gene editing techniques. With appropriate SSc-ILD edits, gene therapies could assist in solving the disease's origin issues.
Our Services
Our company focuses on providing a platform for SSc-ILD investigation and therapeutics development. We can create animal models for you to understand better the experimental SSc-ILD pathophysiology and develop innovative therapies, based on our expertise in model systems and experimental design.
Platforms of SSc-ILD Therapy Development
Animal Models of SSc-ILD
These animal models have been instrumental to understanding the pathogenesis of and developing possible therapies for SSc-ILD. Our company specializes in making these complex animal models more accessible for researchers and other practitioners for SSc-ILD in vivo studies.
| Chemical-induced Models | ||
|---|---|---|
| Bleomycin and hypochlorous acids are chemicals that are commonly used to induce pulmonary fibrosis in animal models. This model mimics the fibrotic changes seen in the lungs of individuals with SSc-ILD. | ||
| Optional Models |
|
|
| Genetically Engineered Models | ||
| Several genetically modified models have been developed to study systemic sclerosis, including models that exhibit skin fibrosis and lung involvement similar to SSc-ILD. | ||
| Optional Models |
|
|
|
||
| Optional Species | Mice, Rats, Others | |
We provide the bleomycin induced model, as well as the genetic engineering model that is designed to study specific molecular pathways related to the target disease. Whatever way you choose, our products focus on assisting you meet your pharmacokinetics and drug safety evaluation needs.
If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
References
- Mirsaeidi, Mehdi, et al. "Systemic Sclerosis Associated Interstitial Lung Disease: New Directions in Disease Management." Frontiers in medicine 6 (2019): 248.
- Makol, Ashima, et al. "Recent innovations in the screening and diagnosis of systemic sclerosis-associated interstitial lung disease." Expert review of clinical immunology 19.6 (2023): 613–626.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.