Kawasaki Disease

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Kawasaki Disease

Kawasaki disease is a systemic vasculitis that occurs in infants and young children and is the most prevalent cause of acquired heart disease in children in developed countries. The most feared consequence of Kawasaki disease is the development of coronary artery abnormalities, which occurs at 20-25% in untreated children. Providing full preclinical research service solutions for rare cardiovascular diseases such as Kawasaki disease, Protheragen is your trusted partner.

Introduction to Kawasaki Disease

Kawasaki disease is an acute febrile illness and systemic vasculitis of unknown etiology that primarily affects infants and young children, causes coronary artery aneurysms, and may lead to late cardiovascular sequelae. Kawasaki disease untreated children develop coronary artery aneurysms which cause ischemic heart disease and sometimes myocardial infarction. There is a substantial ethnic variation with rates highest among the Asian/Pacific Islanders at 29.8/100,000 children aged <5 years and lowest among white children at 13.7/100,000.

The etiology of vasculitis in Kawasaki disease.Fig.1 Pathogenesis of Kawasaki disease vasculitis. (Noval Rivas, M., and Arditi, M., 2020)

Pathogenesis of Kawasaki Disease

Kawasaki disease is characterized by the presence of infiltrates of a wide array of innate and adaptive immune cells in the coronary artery wall. Immunohistochemical studies performed on human autopsies demonstrate the accumulation in the arterial wall of monocytes, macrophages, and, neutrophils, and the presence of activated CD8+ T cells, and IgA+ plasma cells. Release of pro-inflammatory cytokines such as TNF and IL-1β by infiltrating immune cells contributes to vascular endothelial cell injury and the formation of coronary artery aneurysms.

Kawasaki disease is a disease of complex etiology involving both environmental and genetic factors.Fig.2 Environmental and genetic factors. (Noval Rivas, M., and Arditi, M., 2020)

Therapeutics Development for Kawasaki Disease

Drug Name Mechanism of Action Targets NCT Number Research Phase
Rivaroxaban Directly inhibiting Factor Xa, thereby blocking thrombus formation within coronary artery aneurysms and the chance of clot-related events. Factor Xa NCT05643651 Phase IV
Anakinra Suppressing IL-1 signaling by blocking the IL-1 receptor to curb inflammation and mitigate the vasculitis seen in the disease. IL-1Ra NCT04656184 Phase III
Intravenous Immunoglobulin (IVIG) Suppresses the expression of pro-inflammatory cytokines and chemokines, and reduces the activation and quantity of activated T cells. / / Approved
Prednisolone As a corticosteroid that subdues the immune system's inflammatory reaction, ultimately lowering inflammation and averting damage to the coronary arteries. GR NCT04078568 Phase III

Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a therapy plan recommendation. For guidance on therapy options, please visit a regular hospital.

Our Services

Continuing to break the mold in the crusade against rare heart disorders, such as Kawasaki disease, our company is an elite source of fully integrated drug development services. With a comprehensive line of services ranging from diagnostics to therapeutics to disease modeling, we are uniquely positioned to meet the multi-faceted challenges posed by these rare diseases. Using cutting-edge platforms and breakthrough research approaches, we help our clients unlock the true value of drug targets and innovative therapy.

Therapeutic Development Services

Animal Model Development for Kawasaki Disease

The nuances of Kawasaki disease call for accurate and relevant models to dissect its pathophysiology and to test therapies efficiently. Acknowledging the importance of animal model development in drug discovery and Kawasaki disease research, our company provides dedicated, tailor-made services to fulfill this vital requirement.

Induced Animal Models

Generation of induced Kawasaki disease animal models involves the induction of vasculitis and coronary artery inflammation in animals by the administration of certain agents.

  • Lactobacillus casei cell wall extract (LCWE) induced model
  • Candida albicans water-soluble fraction induced model
  • Nod1 ligand (FK565) induced model
  • Other models

Covering all aspects of preclinical development, Protheragen's capabilities include extensive pharmacokinetic studies and a broad range of safety assessments. Leveraging cutting-edge analytical technologies and customized strategic solutions, we provide critical, relevant data needed for making optimal drug development decisions. Please contact us if you'd like to learn more about how we can help you.

Reference

  • Noval Rivas, Magali, and Moshe Arditi. "Kawasaki disease: pathophysiology and insights from mouse models." Nature reviews. Rheumatology 16.7 (2020): 391-405.

For research use only, not for clinical use.