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Systemic Sclerosis-associated Interstitial Lung Disease (SSc-ILD)

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is characterized by excessive collagen production, inflammation, and fibrosis, which poses significant challenges due to its complex nature and potentially devastating impact on individuals' respiratory health. In the realm of rare disease research, our company stands at the forefront with innovative platforms and cutting-edge solutions that can support your research of SSc-ILD pathogenesis and the development of novel therapies.

Introduction to SSc-ILD

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a chronic autoimmune condition characterized by pulmonary inflammation and progressive scarring. Roughly 20 per million adults in the United States are diagnosed with SSc annually, with a striking 90% of cases showing association with interstitial lung disease. SSc-ILD is a special form of ILD, necessitating timely and effective therapeutic to prevent irreversible lung damage and a decline in individuals' quality of life and survival rates. 

Pathogenesis of SSc-ILD

The pathogenesis of SSc-ILD is multifaceted, involving dysregulated immune responses, aberrant fibroblast activation, and impaired wound healing processes. These factors culminate in the aberrant deposition of collagen and other extracellular matrix proteins within lung tissue, resulting in progressive interstitial fibrosis, compromised lung function, and ultimately, respiratory failure (Fig.1).

Fig.1 The pathogenesis of SSc-ILD involves vascular, immunological, and fibrotic processes.Fig.1 The pathogenesis of SSc-ILD involves vascular, immunological, and fibrotic processes. (Mirsaeidi, M., et al., 2019)

Biomarkers for SSc-ILD Diagnostics

Accurate diagnosis and personalized therapeutic strategies are crucial for managing SSc-ILD effectively. To address these challenges, we have proposed the use of potential biomarkers that can aid in the identification of SSc-ILD cases at high risk of 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

Immunosuppressive agents such as cyclophosphamide, mycophenolate mofetil, and Pomalidomide, as well as anti-fibrotic drugs like nintedanib and pirfenidone, have demonstrated efficacy in alleviating symptoms and slowing down disease progression in SSc-ILD individuals.

Cell Therapy

Cell therapy, particularly autologous hematopoietic stem cell transplantation (AHSCT), has also emerged as a potential therapeutic option for severe cases of SSc-ILD. This innovative approach involves using its own stem cells to regenerate and repair damaged lung tissue.

Monoclonal Antibodies Therapy

Monoclonal antibodies have shown promise in targeting specific immune pathways of SSc-ILD. Rituximab, which targets CD20-positive B lymphocytes, and TociIizumab, which targets the human IL-6 receptor alpha chain, are examples of monoclonal antibodies that have been used to treat SSc-ILD individuals.

Gene Therapy

CRISPR-Cas9 can be utilized to target specific genes involved in the dysregulated immune responses and fibrotic processes characteristic of the disease. By correcting genetic abnormalities associated with SSc-ILD, gene editing technologies hold promise for addressing the underlying pathogenesis of the disease.

Our Services

Our company specializes in platform development for SSc-ILD research and therapeutic. By leveraging our proficiency in model creation and experimental design, we can offer you animal models and therapeutics platform development, which aim to enhance your understanding of SSc-ILD pathophysiology and facilitate innovative therapy investigations.

Platforms of SSc-ILD Therapy Development

Animal Models of SSc-ILD

The animal models have been essential in understanding of the pathogenesis and advancing potential therapeutic strategies for SSc-ILD. Our company specializes in providing researchers and professionals with access to these sophisticated animal models, ensuring a robust foundation for in vivo investigations in the realm of SSc-ILD research.

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
  • Bleomycin-induced model
  • Hypochlorous acids-induced model
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
  • Tsk-1/+ model
  • DO11.10 model
  • Fibrillin-1 mutation model
Optional Species Mice, Rats, Others

Whether opting for the bleomycin-induced model, or the genetic engineering model, which allows for targeted exploration of specific molecular pathways implicated in the disease, our offerings cater to your research needs and objectives to support your pharmacokinetics analysis and drug safety evaluation.

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.

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