Adult-onset Still's Disease
Adult-onset Still's disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, fever, skin rash, and elevated ferritin levels. Our company is focused on providing drug and therapeutic development solutions in the field of autoimmune diseases and inflammation, including AoSD.
Introduction to Adult-onset Still's Disease
Adult-onset Still's disease (AoSD) is a complex and enigmatic disorder that continues to challenge our understanding. This condition is characterized by recurrent episodes of fever, joint pain, skin rash, and systemic inflammation. The pathogenesis of AoSD involves the dysregulation of various cytokines, including interleukin-1 (IL-1), interleukin-6 (IL-6), and tumor necrosis factor-alpha (TNF-alpha), which play critical roles in driving the inflammatory process. The innate immune system, particularly cells such as macrophages, assumes a central role in initiating the inflammatory cascade observed in AoSD.
Fig.1 Therapeutics in adult-onset Still's disease (AoSD). (Tomaras S., et al., 2021)
Therapy Discovery and Development for Adult-onset Still's Disease
Targeted biologic therapies have shown promise in managing AoSD by specifically inhibiting key cytokines involved in the disease pathogenesis. These therapies include:
| Therapies | Description |
|---|---|
| Anti-IL-1 Therapy | Anakinra, a recombinant humanized IL-1 receptor antagonist, has been used to treat AoSD, showing rapid improvement in systemic features. |
| Anti-IL-1 Therapy | Canakinumab, a fully human antibody against IL-1β, has also demonstrated improvement in various clinical aspects of AoSD. |
| Anti-IL-6 Therapy | Tocilizumab, a humanized monoclonal antibody against the IL-6 receptor, has shown promising results in improving both systemic and arthritic manifestations of AoSD. |
| JAK Inhibitors | Janus kinase (JAK) inhibitors, such as tofacitinib and baricitinib, block proinflammatory signaling pathways and have shown promise in treating refractory AoSD. |
At our company, we offer a comprehensive range of services for adult-onset Still's Disease diagnostics and therapy development. You can learn more about our therapy development services by clicking on the link below.
Our Services
Our dedicated team of researchers conducts preclinical studies to evaluate novel drug candidates for AoSD. Using animal models and in vitro models, we assess the efficacy and safety of potential therapies, providing valuable insights into their mechanism of action. Our preclinical research services aim to bridge the gap between experimental findings and clinical translation, facilitating the development of promising therapeutics for AoSD.
IL-1Ra Targeted Mutation Models
We provide services for developing mouse models with targeted mutations in the IL-1 receptor antagonist (IL-1Ra) gene. This model exhibits AOSD-like symptoms, including fever, arthritis, and elevated levels of pro-inflammatory cytokines.
Our company has expertise in utilizing cell lines, such as THP-1 and U937, which can be differentiated into macrophage-like cells to study the inflammatory responses in AOSD. These cell-based models allow for high-throughput screening of potential therapeutics and the evaluation of their effects on inflammatory pathways.
By culturing patient-derived stem cells in specific conditions, we can generate joint organoids that recapitulate the architecture and cell types found in the synovial tissue affected by AOSD. Similarly, liver organoids can be generated to study the hepatic manifestations of AOSD, including hepatomegaly and elevated liver enzymes.
Through rigorous testing and optimization, we strive to deliver safe and effective therapies that target key pathways involved in AoSD pathogenesis. Beyond the aforementioned repertoire of services and models, our expertise extends to crafting personalized solutions and designing disease models that impeccably align with your unique needs. If our comprehensive range of offerings has piqued your interest, we wholeheartedly encourage you to connect with us without any hesitation.
Reference
- Tomaras Stylianos, et al. "Adult-onset Still's disease: clinical aspects and therapeutic approach." Journal of clinical medicine 10.4 (2021): 733.