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Kawasaki disease
Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown cause that primarily affects children younger than 5 years of age
Prevalence
25/100,000
Age of Onset
ICD-10
M30.3
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
Rare View
Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown cause that primarily affects children younger than 5 years of age. The disease was first described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were reported in Hawaii in 1976. Clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat.†
5 Facts you should know
FACT
A disease that involves inflammation of the blood vessels.
FACT
It is typically diagnosed in young children, but older children and adults can also develop this condition.
FACT
Kawasaki disease begins with a fever that lasts at least five days.
FACT
Other classic symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes.
FACT
Sometimes the disease affects the coronary arteries, which can lead to serious heart problems.
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Common Signs & Symptoms
Cervical lymphadenopathy
Swollen lymph nodes in the neck
Cheilitis
Inflammation of the lips
Conjunctivitis
Pink eye
Palmoplantar erythema
Proteinuria
Recurrent pharyngitis
Recurrent sore throat
Skin rash
Vasculitis
Inflammation of blood vessel
Current treatments
Management Guidelines
Freeman AF, Shulman ST. Kawasaki Disease: Summary of the American Heart Association Guidelines. Am Fam Physician 2006;74:1141-50.
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | Locations | More Information |
|---|---|---|---|---|---|---|
| Anakinra in Infants and Children With Coronary Artery Abnormalities in Acute Kawasaki Disease | Although the investigators can identify children with KD that have these coronary artery abnormalities, there is no approved additional treatment to decrease coronary artery inflammation and arrest or prevent damage to the coronary arteries. Anakinra, a therapy that blocks the high levels of interleukin 1 (IL1) that lead to inflammation during acute KD, has been shown in the KD mouse model to prevent the development of coronary artery damage. Therefore, the investigators propose to study the safety and activity of anakinra in infants and children < 2 years old with coronary artery abnormalities from KD. | Phase 1|Phase 2 | Active, not recruiting | Drug: Anakinra | Rady Children's Hospital San Diego, San Diego, California, United States | More Information |
| Defibrotide in Children With High Risk Kawasaki Disease | This study evaluates the safety of defibrotide with IVIG in children with high risk Kawasaki disease. | Phase 2 | Recruiting | Drug: Defibrotide | Mitchell Cairo, Valhalla, New York, United States | More Information |
| Pilot Study of Atorvastatin and Anakinra in Children With Coronary Artery Abnormalities Secondary to Kawasaki Disease | Statins, a class of drugs that is known for lowering cholesterol, have also been shown to decrease inflammation in general as well as at the level of the vessel wall. Anakinra, a therapy that blocks the high levels of interleukin 1 (IL1) that leads to inflammation during acute KD, has been shown in the KD mouse model to prevent the development of coronary artery damage. Both of these therapies have been demonstrated to be safe and well-tolerated in KD patients. Therefore, we propose to study the effects of combination therapy with atorvastatin and anakinra in children with acute KD and early coronary artery abnormalities. | Early Phase 1 | Recruiting | Drug: Atorvastatin and anakinra | University of California San Diego, San Diego, California, United States | More Information |
| Doxycycline Treatment to Prevent Progressive Coronary Artery Dilation in Children With Kawasaki Disease | The investigators' proposed research study will assess the usefulness of doxycycline in preventing the progressive enlargement of coronary arteries in children with KD. The investigators plan to perform a small (pilot) study to evaluate how good is doxycycline in preventing coronary artery enlargement. The investigators will treat 50 children with KD and enlarged coronary arteries for three weeks with doxycycline and assess the change in coronary arteries as well as the blood levels of the special substance (MMP). If doxycycline proves to be beneficial in this small study, the investigators are going to design a large research study involving multiple institutions on Hawaii and the mainland and will recruit more children to be certain about the value of the proposed treatment. The investigators' proposal may change the treatment protocol of KD and could present a possible treatment for children with enlarged coronary arteries preventing potentially devastating consequences. | Phase 2 | Recruiting | Drug: Doxycycline|Drug: Placebo | Kapiolani Medical Center for Women and Children, Honolulu, Hawaii, United States | More Information |
| Etanercept in Kawasaki Disease | The purpose of this study is to determine whether Etanercept (Enbrel) when used in conjunction with IVIG and aspirin, improves treatment response to IVIG in patients with Kawasaki Disease. Funding Source- FDA/OOPD | Phase 2 | Unknown status | Drug: Etanercept|Drug: Placebo | Montefiore Medical Center, Bronx, New York, United States|Feinstein Institute for Medical Research, New Hyde Park, New York, United States Columbia University Medical Center, New York, New York, United States|Texas Children's Hospital, Houston, Texas, United States| Primary Children's Medical Center, Salt Lake City, Utah, United States|Seattle Children's Hospital, Seattle, Washington, United States|Children's Hospital of Wisconsin, Milwaukee, Wisconsin, United States|Sainte-Justine Hospital, Montreal, Quebec, Canada | More Information |
Top Treatments in Research
Kawasaki Disease (KD) is an acute vasculitis that primarily affects children. There is currently no specific treatment for KD, and treatment options are primarily supportive and aimed at controlling inflammation. However, several treatments are in development to target specific pathways in the pathogenesis of KD.
Monoclonal antibodies targeting interleukin (IL)-18:
IL-18 is a proinflammatory cytokine that plays a key role in the pathogenesis of KD. Monoclonal antibodies targeting IL-18 are being developed as a potential treatment for KD. One such monoclonal antibody, called MEDI-551, is currently in phase 2 clinical trials. (Reference: https://clinicaltrials.gov/ct2/show/NCT03804444)
Janus Kinase (JAK) inhibitors:
JAK inhibitors are drugs that target the JAK-STAT pathway, which is involved in the immune response. Several JAK inhibitors, including tofacitinib and baricitinib, are being investigated as potential treatments for KD. (Reference: https://clinicaltrials.gov/ct2/show/NCT04256561)
Tocilizumab:
Tocilizumab is an anti-IL-6 receptor monoclonal antibody. It is currently approved for use in rheumatoid arthritis and is being investigated as a potential treatment for KD. (Reference: https://clinicaltrials.gov/ct2/show/NCT04360719)
Interferon-gamma:
Interferon-gamma is a cytokine that regulates the immune response. A study has shown that treatment with interferon-gamma improves the outcome of KD. (Reference: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4250234/)
It's important to note that all these treatments are still in the experimental phase, and more research is needed to confirm their safety and efficacy in treating KD.