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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.



US Estimated

Age of Onset





Autosomal dominant


Autosomal recessive




X-linked dominant


X-linked recessive


5 Facts you should know



A disease that involves inflammation of the blood vessels.



It is typically diagnosed in young children, but older children and adults can also develop this condition.



Kawasaki disease begins with a fever that lasts at least five days.



Other classic symptoms may include red eyes, lips, and mouth; rash; swollen and red hands and feet; and swollen lymph nodes.



Sometimes the disease affects the coronary arteries, which can lead to serious heart problems.

Kawasaki disease

Activated PI3K Delta Syndrome

KD, Mucocutaneous lymph node syndrome

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Common Signs & Symptoms

Cervical lymphadenopathy

Swollen lymph nodes in the neck


Inflammation of the lips


Pink eye

Palmoplantar erythema


High urine protein levels
Protein in urine

Recurrent pharyngitis

Recurrent sore throat

Skin rash


Inflammation of blood vessel

Top clinical studies

TitleDescriptionPhasesStatusInterventionsLocationsMore 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 2Active, not recruitingDrug: AnakinraRady Children's Hospital San Diego, San Diego, California, United StatesMore 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 2RecruitingDrug: DefibrotideMitchell Cairo, Valhalla, New York, United StatesMore 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 1RecruitingDrug: Atorvastatin and anakinraUniversity of California San Diego, San Diego, California, United StatesMore Information
Doxycycline Treatment to Prevent Progressive Coronary Artery Dilation in Children With Kawasaki DiseaseThe 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 2RecruitingDrug: Doxycycline|Drug: PlaceboKapiolani Medical Center for Women and Children, Honolulu, Hawaii, United StatesMore 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/OOPDPhase 2Unknown statusDrug: Etanercept|Drug: PlaceboMontefiore 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 development

AgentClass/Mechanism of ActionDevelopment StatusCompanyCompany ContactClinical StudiesMore Information
leniolisibLeniolisib is a small molecule inhibitor of the delta isoform of the 110 kDa catalytic subunit of class IA PI3K with immunomodulating and potentially anti-neoplastic activities. Leniolisib inhibits the production of phosphatidylinositol-3-4-5-trisphosphate (PIP3). PIP3 serves as an important cellular messenger specifically activating AKT (via PDK1) and regulates a multitude of cell functions such as proliferation, differentiation, cytokine production, cell survival, angiogenesis, and metabolism. Unlike PI3Kα and PI3Kβ which are ubiquitously expressed, PI3Kẟ and PI3Kγ are expressed primarily in cells of hematopoietic origin. The central role of PI3Kẟ in regulating numerous cellular functions of the adaptive immune system (B-cells and to a lesser extent T cells) as well as the innate immune system (neutrophil, mast cells, and macrophages) strongly indicates that PI3Kẟ is a valid and potentially effective therapeutic target for several immune diseases. To date, leniolisib has proven to be safe and well tolerated in healthy subjects as well as the APDS patients during the Phase 1 first-in-human trial and an ongoing open label extension trial.Phase 3Pharming Group N.V.Anurag Relan, MD
Chief Medical Officer
Sirolimus (Rapamycin)The mTOR inhibitor, Sirolimus (Rapamycin) has been found to decrease in non-neoplastic lymphoproliferation. mTOR (mammalian target of rapamycin) is activated downstream of PI3K and has a prominent role in T cell metabolism and the regulation of immune responses. Previously Sirolimus therapy had been reported in a case of APDS to reduce hepatosplenomegaly and lymphadenopathy, increase naïve T cell frequencies, and restore T cell proliferation and IL-2 secretion. Recently Maccari et al. published the initial findings of the ESID APDS registry.Phase 1/2This agent is being studied by Children's Hospital of Fudan Universityn/ahttps://clinicaltrials.gov/ct2/results?cond=APDS&term=rapamycin&cntry=&state=&city=&dist=https://medlineplus.gov/druginfo/meds/a602026.html
NemiralisibThe inhaled PI3Kδ inhibitor, GSK2269557 or Nemiralisib, is also currently being studied in APDS sponsored by GlaxoSmithKline (NCT02593539). Though an oral inhibitor maybe more effective for lymphoproliferation; it is proposed an inhaled inhibitor could benefit patients primarily affected by airway infection and bronchiectasis. The GSK2269557 clinical trial has not as yet reported results, but is described as a “multi-center, non-randomized, open-label, uncontrolled, single group study to investigate the safety and pharmacokinetics during 84 days repeat dosing treatment with 1,000 micrograms of inhaled in addition to standard of care, in subjects with APDS.” GSK2269557 is also currently being investigated as an anti-inflammatory treatment in Chronic Obstructive Pulmonary Disease (COPD).Phase 2GSKn/ahttps://clinicaltrials.gov/ct2/show/NCT02593539https://www.frontiersin.org/articles/10.3389/fimmu.2018.02043/full