Kawasaki Disease (KWD)

Categories: Bone diseases, Cardiovascular diseases, Immune diseases, Rare diseases

Aliases & Classifications for Kawasaki Disease

MalaCards integrated aliases for Kawasaki Disease:

Name: Kawasaki Disease 57 12 20 43 58 72 36 54 42 3 15 62
Mucocutaneous Lymph Node Syndrome 57 12 20 43 58 72 44 17 70
Acute Febrile Mucocutaneous Lymph Node Syndrome 12 43 29
Kawasaki Disease, Susceptibility to 13 6 39
Infantile Polyarteritis 57 72 70
Kawasaki Syndrome 73 20 43
Kd 57 43 3
Acute Febrile Mcls 12
Kawasaki's Disease 12
Mlns 12
Kwd 72


Orphanet epidemiological data:

kawasaki disease
Inheritance: Multigenic/multifactorial; Age of onset: Adolescent,Adult,Childhood,Infancy; Age of death: normal life expectancy;


Orphanet: 58  
Rare circulatory system diseases
Rare systemic and rhumatological diseases

Summaries for Kawasaki Disease

MedlinePlus : 42 What is Kawasaki disease? Kawasaki disease is a rare illness that usually affects small children. Other names for it are Kawasaki syndrome and mucocutaneous lymph node syndrome. It is a type of vasculitis, which is inflammation of the blood vessels. Kawasaki disease is serious, but most children can fully recover if they are treated right away. What causes Kawasaki disease? Kawasaki disease happens when the immune system injures the blood vessels by mistake. Researchers do not fully know why this happens. But when it does, the blood vessels become inflamed and can narrow or close off. Genetics may play a role in Kawasaki disease. There may also be environmental factors, such as infections. It does not seem to be contagious. This means that it cannot be passed from one child to another. Who is at risk for Kawasaki disease? Kawasaki disease usually affects children under the age of 5. But older children and adults can sometimes get it. It is more common in boys than girls. It can affect children of any race, but those with Asian or Pacific Islander descent are more likely to get it. What are the symptoms of Kawasaki disease? The symptoms of Kawasaki disease may include High fever lasting at least five days A rash, often on the back, chest, and groin Swollen hands and feet Redness of the lips, lining of the mouth, tongue, palms of the hand, and soles of the feet Pink eye Swollen lymph nodes What other problems can Kawasaki disease cause? Sometimes Kawasaki disease can affect the walls of the coronary arteries. These arteries bring supply blood and oxygen to your heart. This can lead to An aneurysm (bulging and thinning of the walls of the arteries). This can raise the risk of blood clotss in the arteries. If the blood clots are not treated, they could lead to a heart attack or internal bleeding. Inflammation in the heart Heart valve problems Kawasaki disease can also affect other parts of the body, including the brain and nervous system, the immune system, and the digestive system. How is Kawasaki disease diagnosed? There is no specific test for Kawasaki disease. To make a diagnosis, your child's health care provider will do a physical exam and look at the signs and symptoms. The provider will likely do blood and urine tests to rule out other diseases and check for signs of inflammation. He or she may do tests to check for damage to the heart, such as an echocardiogram and electrocardiogram (EKG). What are the treatments for Kawasaki disease? Kawasaki disease is usually treated in the hospital with an intravenous (IV) dose of immunoglobulin (IVIG). Aspirin may also be part of the treatment. But do not give your child aspirin unless the health care provider tells you to. Aspirin can cause Reye syndrome in children. This is a rare, serious illness that can affect the brain and liver. Usually treatment works. But if it is not working well enough, the provider may also give your child other medicines to fight the inflammation. If the disease affects your child's heart, he or she might need additional medicines, surgery, or other medical procedures.

MalaCards based summary : Kawasaki Disease, also known as mucocutaneous lymph node syndrome, is related to exanthem and conjunctivitis, and has symptoms including fever, congestion of ocular conjunctivae and reddening of lips. An important gene associated with Kawasaki Disease is THRIL (TNF And HNRNPL Related Immunoregulatory Long Non-Coding RNA), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Anti-Inflammatory Agents, Non-Steroidal and Fibrinolytic Agents have been mentioned in the context of this disorder. Affiliated tissues include body, lymph node and heart, and related phenotypes are proteinuria and cheilitis

Disease Ontology : 12 A lymphadenitis characterized by swelling of cervical lymph nodes in infants and young children and inflammation of medium-sized blood vessels located in body, has symptom fever, has symptom congestion of ocular conjunctivae, has symptom reddening of lips, has symptom reddening of oral cavity, has symptom protuberance of tongue papillae and has symptom edema of extremities.

MedlinePlus Genetics : 43 Kawasaki disease is a sudden and time-limited (acute) illness that affects infants and young children. Affected children develop a prolonged fever lasting several days, a skin rash, and swollen lymph nodes in the neck (cervical lymphadenopathy). They also develop redness in the whites of the eyes (conjunctivitis) and redness (erythema) of the lips, lining of the mouth (oral mucosa), tongue, palms of the hands, and soles of the feet.Without treatment, 15 to 25 percent of individuals with Kawasaki disease develop bulging and thinning of the walls of the arteries that supply blood to the heart muscle (coronary artery aneurysms) or other damage to the coronary arteries, which can be life-threatening.

GARD : 20 Kawasaki disease is 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 carry oxygen-rich blood to the heart, which can lead to serious heart problems. Kawasaki disease occurs most often in people of Asian and Pacific Island descent. The cause of Kawasaki disease is unknown. An infection along with genetic factors may be involved. Treatment includes intravenous gamma globulin and high doses of aspirin in a hospital setting. Prognosis is generally very good, but in cases of heart complications it depends on the severity of the coronary disease.

OMIM® : 57 Kawasaki disease is an acute, self-limited vasculitis of infants and children characterized by prolonged fever unresponsive to antibiotics, polymorphous skin rash, erythema of the oral mucosa, lips, and tongue, erythema of the palms and soles, bilateral conjunctival injection, and cervical lymphadenopathy (Kawasaki, 1967). Coronary artery aneurysms develop in 15 to 25% of those left untreated (11,12:Kato et al., 1975, 1996), making Kawasaki disease the leading cause of acquired heart disease among children in developed countries. Treatment with intravenous immunoglobulin (IVIg) abrogates the inflammation in approximately 80% of affected individuals and reduces the aneurysm rate to less than 5%. Cardiac sequelae of the aneurysms include ischemic heart disease, myocardial infarction, and sudden death. Epidemiologic features such as seasonality and clustering of cases suggested an infectious trigger, although no pathogen had been isolated. Several lines of evidence suggested the importance of genetic factors in disease susceptibility and outcome. First, the incidence of Kawasaki disease is 10 to 20 times higher in Japan than in Western countries (Cook et al., 1989). Second, the risk of Kawasaki disease in sibs of affected children is 10 times higher than in the general population, and the incidence of Kawasaki disease in children born to parents with a history of Kawasaki disease is twice as high as that in the general population (Fujita et al., 1989; Uehara et al., 2003). Hata and Onouchi (2009) reviewed current knowledge on Kawasaki disease, including epidemiology, genomewide linkage analysis, and molecular genetics. (611775) (Updated 05-Apr-2021)

CDC : 3 Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown etiology 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.

KEGG : 36 Kawasaki disease (KD) is an acute systemic vasculitis of childhood that does not have a known cause or aetiology. KD is a self-limited illness that is not associated with the production of autoantibodies or the deposition of immune complexes, and it rarely recurs. The disease is believed to result from an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. Classic (typical) Kawasaki disease is diagnosed based on the presence of a fever lasting five or more days, accompanied by four out of five findings: bilateral conjunctival injection, oral changes such as cracked and erythematous lips and strawberry tongue, cervical lymphadenopathy, extremity changes such as erythema or palm and sole desquamation, and polymorphous rash. Incomplete (atypical) Kawasaki disease occurs in persons with fever lasting five or more days and with two or three of these findings. The standard treatment of acute KD is intravenous immunoglobulin (IVIG) infusion and aspirin. However, 10-20% of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis. If there is no response to treatment, patients are given a second dose of IVIG with or without corticosteroids or other adjunctive treatment.

UniProtKB/Swiss-Prot : 72 Kawasaki disease: An acute, self-limited vasculitis of infants and children characterized by prolonged fever unresponsive to antibiotics, polymorphous skin rash, erythema of the oral mucosa, lips, and tongue, erythema of the palms and soles, bilateral conjunctival injection, and cervical lymphadenopathy.

PubMed Health : 62 About kawasaki disease: Kawasaki (KAH-wah-SAH-ke) disease is a rare childhood disease. It's a form of a condition called vasculitis (vas-kyu-LI-tis). This condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout the body become inflamed. The disease can affect any type of blood vessel in the body, including the arteries, veins, and capillaries. Sometimes Kawasaki disease affects the coronary arteries, which carry oxygen-rich blood to the heart. As a result, some children who have Kawasaki disease may develop serious heart problems.

Wikipedia : 73 Kawasaki disease is a syndrome of unknown cause that results in a fever and mainly affects children... more...

Related Diseases for Kawasaki Disease

Diseases related to Kawasaki Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 912)
# Related Disease Score Top Affiliating Genes
1 exanthem 32.0 TNF IL6 GPT CRP
2 conjunctivitis 31.8 TNF MMP9 IL6 CCL2
3 myocarditis 31.7 TNF NPPB IL6 CCL2
4 coronary stenosis 31.7 NPPB IL6 CRP
5 vasculitis 31.5 TNF SELL SELE MBL2 IL6 HSPD1
6 coronary aneurysm 31.5 TNF MMP9 MMP3 ITPKC IL6 GPT
7 pyuria 31.5 ITPKC IL6 GPT CRP
8 purpura 31.4 TNF IL6 CRP
9 pericardial effusion 31.4 NPPB IL6 GPT CRP
10 toxic shock syndrome 31.3 TNF IL6 HSPD1 CRP
11 severe acute respiratory syndrome 31.3 TNF MBL2 IL6 CRP CCL2
12 rheumatic fever 31.3 TNF NPPB MBL2 IL6 FCGR2A
13 scarlet fever 31.2 TNF ITPKC CRP
14 iridocyclitis 31.2 TNF IL6 CRP
15 pharyngitis 31.2 TNF IL6 GPT CRP
16 pericarditis 31.2 TNF NPPB IL6 CRP
17 cardiogenic shock 31.1 TNF NPPB IL6
18 acute myocardial infarction 31.1 SELE NPPB MMP9 IL6 CRP
19 acute myocarditis 31.0 TNF NPPB CRP
20 lymphadenitis 31.0 TNF MYD88 MIR145 ITPKC CRP
21 cellulitis 31.0 TNF IL6 GPT CRP
22 chickenpox 31.0 TNF IL6 CRP
23 congestive heart failure 31.0 TNF NPPB IL6 CRP
24 bacterial conjunctivitis 31.0 TNF ITPKC IL6 CRP
25 endocarditis 31.0 TNF IL6 HSPD1 FCGR2A CRP
26 tricuspid valve insufficiency 31.0 NPPB GPT CRP
27 angina pectoris 31.0 NPPB IL6 CRP
28 aortitis 31.0 TNF IL6 CRP
29 juvenile rheumatoid arthritis 31.0 TNF S100A12 MMP3 IL6 CRP CCL2
30 uveitis 31.0 TNF IL6 HSPD1 CCL2
31 infective endocarditis 30.9 TNF IL6 FCGR2A CRP
32 meningoencephalitis 30.9 TNF IL6 CRP
33 disease by infectious agent 30.9 TNF MBL2 IL6 HSPD1 GPT FCGR2A
34 severe cutaneous adverse reaction 30.8 TNF GPT CRP
35 autoimmune vasculitis 30.8 TNF IL6 ELANE CRP
36 cholangitis 30.8 TNF IL6 GPT CRP
37 pancreatitis 30.8 TNF IL6 ELANE CRP
38 deficiency anemia 30.8 TNF TIMP1 IL6 GPT CRP
39 intestinal obstruction 30.8 TNF IL6 GPT CRP
40 ileus 30.8 TNF IL6 CRP CCL2
41 familial mediterranean fever 30.8 TNF S100A12 IL6 CRP
42 urticaria 30.8 TNF SELE IL6 CRP
43 hydrops of gallbladder 30.7 NPPB ITPKC GPT FCGR2A CRP
44 rheumatic heart disease 30.7 TNF NPPB MBL2 IL6
45 polyarteritis nodosa 30.7 SELL SELE IL6 GPT CRP
46 arteries, anomalies of 30.7 TNF MIR145 IL6 CRP CCL2
47 mycoplasma pneumoniae pneumonia 30.7 TNF IL6 CRP
48 meningitis 30.7 TNF MMP9 MBL2 IL6 FCGR2A CRP
49 acalculous cholecystitis 30.7 GPT CRP
50 obstructive jaundice 30.7 TNF IL6 GPT CRP

Graphical network of the top 20 diseases related to Kawasaki Disease:

Diseases related to Kawasaki Disease

Symptoms & Phenotypes for Kawasaki Disease

Human phenotypes related to Kawasaki Disease:

58 31 (show all 38)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 proteinuria 58 31 hallmark (90%) Very frequent (99-80%) HP:0000093
2 cheilitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0100825
3 recurrent pharyngitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0100776
4 conjunctivitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000509
5 skin rash 58 31 hallmark (90%) Very frequent (99-80%) HP:0000988
6 vasculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002633
7 cervical lymphadenopathy 58 31 hallmark (90%) Very frequent (99-80%) HP:0025289
8 palmoplantar erythema 58 31 hallmark (90%) Very frequent (99-80%) HP:0025493
9 arthritis 58 31 frequent (33%) Frequent (79-30%) HP:0001369
10 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
11 fever 58 31 frequent (33%) Frequent (79-30%) HP:0001945
12 abnormal heart valve morphology 58 31 frequent (33%) Frequent (79-30%) HP:0001654
13 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
14 pericarditis 58 31 frequent (33%) Frequent (79-30%) HP:0001701
15 glossitis 58 31 frequent (33%) Frequent (79-30%) HP:0000206
16 diarrhea 58 31 frequent (33%) Frequent (79-30%) HP:0002014
17 edema 58 31 frequent (33%) Frequent (79-30%) HP:0000969
18 leukocytosis 58 31 frequent (33%) Frequent (79-30%) HP:0001974
19 abnormality of nail color 58 31 frequent (33%) Frequent (79-30%) HP:0100643
20 nausea and vomiting 58 31 occasional (7.5%) Occasional (29-5%) HP:0002017
21 ptosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000508
22 cranial nerve paralysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0006824
23 congestive heart failure 58 31 occasional (7.5%) Occasional (29-5%) HP:0001635
24 hepatitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012115
25 irritability 58 31 occasional (7.5%) Occasional (29-5%) HP:0000737
26 jaundice 58 31 occasional (7.5%) Occasional (29-5%) HP:0000952
27 arthralgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002829
28 arrhythmia 58 31 occasional (7.5%) Occasional (29-5%) HP:0011675
29 migraine 58 31 occasional (7.5%) Occasional (29-5%) HP:0002076
30 myocarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012819
31 meningitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001287
32 cholecystitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001082
33 double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0011658
34 sterile pyuria 58 31 occasional (7.5%) Occasional (29-5%) HP:0100586
35 ascending tubular aorta aneurysm 31 occasional (7.5%) HP:0004970
36 abnormal pulmonary interstitial morphology 31 occasional (7.5%) HP:0006530
37 dilatation of the ascending aorta 58 Occasional (29-5%)
38 interstitial pulmonary abnormality 58 Occasional (29-5%)

Clinical features from OMIM®:

611775 (Updated 05-Apr-2021)


  • fever
  • congestion of ocular conjunctivae
  • reddening of lips
  • reddening of
  • protuberance of tongue papillae
  • edema of extremities

UMLS symptoms related to Kawasaki Disease:

angina pectoris; chest pain; pruritus; edema; exanthema

GenomeRNAi Phenotypes related to Kawasaki Disease according to GeneCards Suite gene sharing:

# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.17 CCL2 FCGR2A HSPD1 MYD88 S100A12 SELE

MGI Mouse Phenotypes related to Kawasaki Disease:

# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.07 ELANE FCGR2A HSPD1 IL6 ITPKC MBL2
2 immune system MP:0005387 10 CCL2 CRP ELANE FCGR2A IL6 ITPKC
3 neoplasm MP:0002006 9.5 ELANE IL6 MMP9 MYD88 SELL TIMP1
4 respiratory system MP:0005388 9.23 HSPD1 IL6 MMP9 MYD88 SELE SELL

Drugs & Therapeutics for Kawasaki Disease

PubMed Health treatment related to Kawasaki Disease: 62

Medicines are the main treatment for Kawasaki disease . Rarely, children whose coronary (heart ) arteries are affected may need medical procedures or surgery . The goals of treatment include: Reducing fever and inflammation to improve symptoms Preventing the disease from affecting the coronary arteries

Drugs for Kawasaki Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 188)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Anti-Inflammatory Agents, Non-Steroidal Phase 4
2 Fibrinolytic Agents Phase 4
3 Cyclooxygenase Inhibitors Phase 4
4 Antipyretics Phase 4
5 Immunoglobulin G Phase 4
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
tannic acid Approved Phase 3 1401-55-4
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
14 Radiopharmaceuticals Phase 3
15 Technetium Tc 99m Sestamibi Phase 3
16 Immunoglobulins, Intravenous Phase 3
17 Immunoglobulins Phase 3
18 Antibodies Phase 3
19 Neuroprotective Agents Phase 3
20 Methylprednisolone Acetate Phase 3
21 Protective Agents Phase 3
22 Interleukin 1 Receptor Antagonist Protein Phase 3
L-Alanine Nutraceutical Phase 3 56-41-7 5950
Etanercept Approved, Investigational Phase 2 185243-69-0
Atorvastatin Approved Phase 1, Phase 2 134523-00-5 60823
Defibrotide Approved, Investigational Phase 2 83712-60-1
Doxycycline Approved, Investigational, Vet_approved Phase 2 564-25-0 54671203
Sodium citrate Approved, Investigational Phase 2 68-04-2
BCG vaccine Approved, Investigational Phase 2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
Ixazomib Approved, Investigational Phase 2 1072833-77-2
Lenalidomide Approved Phase 2 191732-72-6 216326
Citric acid Approved, Nutraceutical, Vet_approved Phase 2 77-92-9 311
Glycine Approved, Nutraceutical, Vet_approved Phase 2 56-40-6 750
Glutamic acid Approved, Nutraceutical Phase 2 56-86-0 33032
37 Antibiotics, Antitubercular Phase 1, Phase 2
38 Antimalarials Phase 2
39 Anti-Bacterial Agents Phase 2
40 Antiparasitic Agents Phase 2
41 Antiprotozoal Agents Phase 2
42 Anti-Infective Agents Phase 2
43 Neurotransmitter Agents Phase 2
44 Citrate Phase 2
45 Adjuvants, Immunologic Phase 2
46 Vaccines Phase 2
47 Antitubercular Agents Phase 2
48 Immunologic Factors Phase 2
49 Antiemetics Phase 2
50 Hormone Antagonists Phase 2

Interventional clinical trials:

(show top 50) (show all 52)
# Name Status NCT ID Phase Drugs
1 Multicenter, Single-arm, Phase IV Study to Evaluate the Efficacy, Safety of Combined Therapy of Aspirin and High Dose "IVIG-SN" in Pediatric Patients With Kawasaki Disease Completed NCT01524939 Phase 4 immunoglobulin G
2 Multicenter, Single-arm, Phase IV Study to Evaluate the Efficacy, Safety of Combined Therapy of Aspirin and IVIG-SN 10% in Pediatric Patients With Kawasaki Disease Completed NCT04003844 Phase 4 Immunoglobulin G;Acetylsalicylic acid
3 A Randomized Open-label Trial to Evaluate the Efficacy of Immunoglobulin Plus Steroid for Prevention of Coronary Artery Abnormalities in Taiwanese Refractory Kawasaki Disease (RAST Study) Unknown status NCT03200561 Phase 3 Prednisolone
4 Trial of Pulse Steroid Therapy in Kawasaki Disease (A Trial Conducted by the Pediatric Heart Network) Completed NCT00132080 Phase 3 Steroids
5 KIDCARE (Kawasaki Disease Comparative Effectiveness Trial) Completed NCT03065244 Phase 3 IVIG;Infliximab
6 Infliximab for Kawasaki Disease Patients Resistant to Intravenous Immunoglobulin : a Multicentre, Prospective, Randomised Trial Completed NCT02298062 Phase 3 Infliximab;IVIG
7 A Phase III, Open-Label, Non-Randomized, International, Multicenter Trial to Evaluate the Efficacy and Safety of CARDIOLITE® Myocardial Perfusion Imaging in Pediatric Subjects With Kawasaki Disease Completed NCT00162032 Phase 3 Sestamibi
8 To Evaluate the Efficacy and Safety of TA-650 in Comparison With a Control Drug Polyethylene Glycol-treated Human Immunoglobulin (VGIH) in Patients With Kawasaki Disease Refractory to Initial Therapy With Intravenous Immunoglobulin (IVIG). Completed NCT01596335 Phase 3 TA-650;Polyethylene Glycol-treated Human Immunoglobulin (VGIH)
9 Infliximab (Remicade®) Plus Intravenous Immunoglobulin (IVIG) for the Primary Treatment of Patients With Acute Kawasaki Disease Completed NCT00760435 Phase 3 Infliximab;Placebo
10 Different Doses of IVIG for Kawasaki Disease: a Multicentre, Prospective, Randomised Trial Completed NCT02439996 Phase 3 IVIG (1g/kg,once);IVIG (1g/kg,twice);IVIG (2g/kg.once)
11 A Clinical Investigation to Assess the Safety and Efficacy of Immune Globulin Intravenous (Human), 10% in Subjects With Primary Immunodeficiency Disorders Completed NCT00157079 Phase 3
12 Efficacy of Immunoglobulin Plus Prednisolone in Reducing Coronary Artery Lesion in Patients With Kawasaki Disease: A Multicentre Randomised Controlled Trial Recruiting NCT04078568 Phase 3 IVIG;Aspirin;Prednisolone
13 A Randomized Phase III Multicenter Trial Comparing the Efficacy and Safety of Anakinra Versus Intravenous Immunoglobulin (IVIG) Retreatment, in Patients With Kawasaki Disease Who Failed to Respond to Initial Standard IVIG Treatment Not yet recruiting NCT04656184 Phase 3 ANAKINRA;Intravenous immunoglobulin
14 Randomised Evaluation of COVID-19 Therapy (RECOVERY) in Children With PIMS-TS in Switzerland (SWISSPED-RECOVERY) Not yet recruiting NCT04826588 Phase 3 Methylprednisolone sodium succinate
15 Efficacy of Primary Treatment With Immunoglobulin Plus Infliximab for the Early Regression of Coronary Artery Lesion in Kawasaki Disease: a Multicenter, Open-label, Blinded-end Randomized Controlled Study. Withdrawn NCT04535518 Phase 3 IVIG;Aspirin;Infliximab
16 A Randomized, Double Blind, Placebo Controlled Study of the Effects of Etanercept in Children Presenting With Kawasaki Disease Unknown status NCT00841789 Phase 2 Etanercept;Placebo
17 An Open Label, Non-comparative Phase II Trial to Evaluate the Effects of Atorvastatin on the Persistent Coronary Arterial Aneurysm in Children With Kawasaki Disease: Safety and Efficacy Unknown status NCT02114099 Phase 2 Atorvastatin
18 Safety and Efficacy Evaluation of Tissue Engineered Construct Based on Allogeneic Adipose-derived Multipotent Mesenchymal Stromal Cells and Platelet-poor Plasma Fibrin Hydrogel to Treat the Patients With Burn Wounds Unknown status NCT03113747 Phase 1, Phase 2
19 Phase I/IIa Study of Pharmacokinetics and Safety of Atorvastatin in Children With Coronary Artery Abnormalities Secondary to Kawasaki Disease Completed NCT01431105 Phase 1, Phase 2 Atorvastatin
20 A Phase IIa Multicenter Trial to Assess the Efficacy, and Safety of Anakinra in Patients With Intravenous Immunoglobulin-resistant Kawasaki Disease Completed NCT02390596 Phase 2 Anakinra
21 A Phase I-II, Open-Label, Multicenter Trial to Determine the Dosimetry and Safety of Technetium Tc99m Sestamibi in Pediatric Subjects Completed NCT00162045 Phase 1, Phase 2 Technetium Tc99m Sestamibi
22 Prevention of Coronary Aneurysms in Kawasaki Syndrome Completed NCT00000520 Phase 2 immunoglobulins, intravenous;aspirin
23 A Pilot Study to Determine the Safety of Defibrotide in Children With High Risk Kawasaki Disease Recruiting NCT04777422 Phase 2 Defibrotide
24 Anakinra in Infants and Children With Coronary Artery Abnormalities in Acute Kawasaki Disease Recruiting NCT02179853 Phase 1, Phase 2 Anakinra
25 Phase 2 Study to Assess the Efficacy and Safety of Doxycycline in Preventing Coronary Artery Aneurysm Formation and Progression Recruiting NCT01917721 Phase 2 Doxycycline;Placebo
26 Phase II Study of the Combination of MLN 9708 With Lenalidomide as Maintenance Therapy Post Autologous Stem Cell Transplant in Patients With Multiple Myeloma Active, not recruiting NCT01718743 Phase 2 Ixazomib Citrate;Lenalidomide
27 Bacille Calmette-Guérin (BCG) Vaccine In Radiologically Isolated Syndrome (RIS) Not yet recruiting NCT03888924 Phase 2 Bacille Calmette-Guerin vaccine;Placebo
28 Evaluation of Ixazomib, Lenalidomide, Dexamethasone Induction and Extended Consolidation Followed by Lenalidomide Maintenance in Newly Diagnosed Multiple Myeloma Patients ≤65 Years Eligible for High Dose Therapy Terminated NCT02897830 Phase 2 Ixazomib;Lenalidomide;Dexamethasone
29 Study of Efficacy and Safety of Canakinumab in Pediatric Patients With Kawasaki Disease Withdrawn NCT02980263 Phase 2 Canakinumab
30 Cardiovascular Risk Markers Before and After Therapy With Statins in Patients With History of Kawasaki Disease Withdrawn NCT00305201 Phase 2 pravastatin
31 Clinical Study of the Efficacy and Safety of the Application of Allogeneic Mesenchymal (Stromal) Cells of Bone Marrow, Cultured Under the Hypoxia in the Treatment of Patients With Severe Pulmonary Emphysema Withdrawn NCT01849159 Phase 1, Phase 2
32 Infliximab (Remicade) for Patients With Acute Kawasaki Disease Who Fail to Become Afebrile After Intravenous Gamma Globulin Therapy Completed NCT00271570 Phase 1 Infliximab (Remicade)
33 Phase I Study of MLN 9708 in Addition to Chemotherapy for the Treatment of Acute Lymphoblastic Leukemia in Older Adults Completed NCT02228772 Phase 1 MLN 9708;Vincristine;Cytarabine;Doxorubicin;Mercaptopurine;Cyclophosphamide;Methotrexate
34 Methylprednisolone Pulse Therapy for Coronary Artery Dilatation or Aneurysm Formation in Kawasaki Disease Recruiting NCT04509219 Phase 1 Methylprednisolone Injection
35 Multi-center Prospective Randomized Control Trail of High Dose Aspirin in Acute Stage of Kawasaki Disease Unknown status NCT02359643 Aspirin
36 Measurement of Antibodies in Adults With a History of Kawasaki Disease Unknown status NCT02853266
37 Etiology Study of Kawasaki Disease--A Prospective Household and Case Control Study Unknown status NCT00154596
38 Safety and Vascular Remodelling After Bioresorbable Vascular Scaffold Implantation for Stenotic or Occluded Lesions in Children and Young Adults With Kawasaki Disease Unknown status NCT02771288
39 Kawasaki Disease Center, Kaohsiung Chang Gung Memorial Hospital, Taiwan Unknown status NCT02951234
40 Epidemiologic Features of Kawasaki Disease in Shanghai From 2013 Through 2017 Completed NCT03880929
41 Prevalence and Early Markers of Atherosclerosis in Adults With a History of Kawasaki Disease Completed NCT01440075
42 Acute Abdomen in Kawasaki Disease: Case Reports Completed NCT03902262
43 Epidemiologic Features of Kawasaki Disease in Shanghai From 2008 Through 2012 Completed NCT02317913
44 Quantification of Ventricular Mechanics/Myocardial Blood Flow Reserve in Adolescents and Adults Ages 12-50 With Congenital or Acquired Heart Disease Using Bicycle Stress With Real Time Myocardial Contrast Echo and Dobutamine Stress MRI Completed NCT00861848
45 Reproductive Health in Men and Women With Vasculitis Completed NCT02176070
46 Application of Statins in Long-term Treatments of Kawasaki Disease Complicated With Severe Coronary Artery Abnormalities in Children Recruiting NCT03915795
47 Pilot Study of Atorvastatin and Anakinra in Children With Coronary Artery Abnormalities Secondary to Kawasaki Disease Recruiting NCT04747847 Early Phase 1 Atorvastatin and anakinra
48 Genetic Determinants of Kawasaki Disease for Susceptibility and Outcome Recruiting NCT03861130
49 Characterization of Multisystem Inflammatory Syndrome in Children (MIS-C) and Its Relationship to Kawasaki Disease Recruiting NCT04538495
50 Cardiovascular Status of Children 5 Years After Kawasaki Disease Recruiting NCT03750123

Search NIH Clinical Center for Kawasaki Disease

Cochrane evidence based reviews: mucocutaneous lymph node syndrome

Genetic Tests for Kawasaki Disease

Genetic tests related to Kawasaki Disease:

# Genetic test Affiliating Genes
1 Acute Febrile Mucocutaneous Lymph Node Syndrome 29

Anatomical Context for Kawasaki Disease

The Foundational Model of Anatomy Ontology organs/tissues related to Kawasaki Disease:


MalaCards organs/tissues related to Kawasaki Disease:

Lymph Node, Heart, Tongue, Endothelial, Neutrophil, Skin, T Cells

Publications for Kawasaki Disease

Articles related to Kawasaki Disease:

(show top 50) (show all 6918)
# Title Authors PMID Year
ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms. 6 57 61
18084290 2008
GM-CSF primes cardiac inflammation in a mouse model of Kawasaki disease. 57 61
27595596 2016
Two new susceptibility loci for Kawasaki disease identified through genome-wide association analysis. 57 61
22446961 2012
A genome-wide association study identifies three new risk loci for Kawasaki disease. 61 57
22446962 2012
Genome-wide association study identifies FCGR2A as a susceptibility locus for Kawasaki disease. 57 61
22081228 2011
ITPKC gene SNP rs28493229 and Kawasaki disease in Taiwanese children. 61 57
20045869 2010
Susceptibility genes for Kawasaki disease: toward implementation of personalized medicine. 57 61
19158812 2009
A genomewide linkage analysis of Kawasaki disease: evidence for linkage to chromosome 12. 57 61
17160344 2007
Gene expression profiling of the effect of high-dose intravenous Ig in patients with Kawasaki disease. 57 61
15843588 2005
CD40 ligand gene and Kawasaki disease. 57 61
15367912 2004
Kawasaki disease in parents and children. 61 57
12856980 2003
Expression of CD40 ligand on CD4+ T-cells and platelets correlated to the coronary artery lesion and disease progress in Kawasaki disease. 57 61
12563087 2003
Two-generation Kawasaki disease: mother and daughter. 61 57
11713463 2001
Kawasaki disease in a father and daughter. 57 61
10447146 1999
Long-term consequences of Kawasaki disease. A 10- to 21-year follow-up study of 594 patients. 61 57
8822996 1996
Results from an international survey of Kawasaki disease in 1979-82. 61 57
2605549 1989
Kawasaki disease in families. 61 57
2780128 1989
Coronary aneurysms in infants and young children with acute febrile mucocutaneous lymph node syndrome. 57 61
236368 1975
A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. 61 57
4153258 1974
Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey. 61 42
33726806 2021
Phenotype, Susceptibility, Autoimmunity, and Immunotherapy Between Kawasaki Disease and Coronavirus Disease-19 Associated Multisystem Inflammatory Syndrome in Children. 61 42
33732254 2021
Potential link between SARS-CoV-2 and Kawasaki disease: importance of dentists for the diagnosis. 61 42
33729297 2021
Familial occurrence of Kawasaki syndrome in North America. 57
16143748 2005
Kawasaki syndrome. 57
15302199 2004
An epidemic of Kawasaki syndrome in Hawaii. 57
7062202 1982
Kawasaki syndrome: description of two outbreaks in the United States. 57
7231502 1981
[Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. 57
6062087 1967
[Levels of serum brain natriuretic peptide and the correlation to heart function in children with Kawasaki disease]. 61 54
20350422 2010
Effect of angiotensin-converting enzyme inhibitor on matrix metalloproteinase-9 activity in patients with Kawasaki disease. 61 54
19945447 2010
[Roles of matrix metalloproteinases, tissue inhibitor of matrix metalloproteinase and hs-CRP in Kawasaki disease]. 54 61
20113606 2009
Association of mannose-binding lectin gene polymorphisms with Kawasaki disease in the Japanese. 61 54
20374367 2009
[Effect of small interfering RNA on matrix metalloproteinase-9 expression in vascular endothelial cells stimulated by serum from children with Kawasaki disease]. 61 54
20128385 2009
Inhibition of matrix metalloproteinase-9 activity improves coronary outcome in an animal model of Kawasaki disease. 54 61
19604270 2009
Amino-terminal pro-B-type natriuretic peptide in refractory Kawasaki disease. 61 54
19365663 2009
Brain natriuretic peptide levels in Kawasaki disease: a case report. 61 54
19500284 2009
Association of the matrix metalloproteinase-3 (-439C/G) promoter polymorphism with Kawasaki disease in Korean children. 61 54
18810583 2008
Inflammatory gene polymorphisms and susceptibility to kawasaki disease and its arterial sequelae. 61 54
18710885 2008
Matrix metalloproteinase 9 activity leads to elastin breakdown in an animal model of Kawasaki disease. 54 61
18311803 2008
[Biochemical and immunological laboratory findings in Kawasaki disease]. 61 54
18260330 2008
[Expression of monocyte chemotactic protein-1 in peripheral blood mononuclear cells of children with Kawasaki disease and its relation to coronary artery impairment]. 54 61
19099689 2008
[Reducing plasma homocysteic acid lowers serum C-reactive protein level in children with Kawasaki disease]. 61 54
18024309 2007
Persistent monocytosis after intravenous immunoglobulin therapy correlated with the development of coronary artery lesions in patients with Kawasaki disease. 61 54
17932598 2007
Levels of intra- and extracellular heat shock protein 60 in Kawasaki disease patients treated with intravenous immunoglobulin. 54 61
17602873 2007
Serum MMP-2 and MMP-9 in patients with Behçet's disease: do their higher levels correlate to vasculo-Behçet's disease associated with aneurysm formation? 61 54
17949555 2007
Increased CD11b expression on polymorphonuclear leucocytes and cytokine profiles in patients with Kawasaki disease. 61 54
17286760 2007
Abnormal tissue doppler images are associated with elevated plasma brain natriuretic peptide and increased oxidative stress in acute Kawasaki disease. 61 54
17322635 2007
Prediction of non-responsiveness to standard high-dose gamma-globulin therapy in patients with acute Kawasaki disease before starting initial treatment. 61 54
16896641 2007
[Change in plasma N-terminal pro-brain natriuretic peptide in children with Kawasaki disease and its value in clinical practice]. 54 61
17254452 2006
Increased serum levels of S100A12 in patients with MPO-ANCA-associated glomerulonephritis. 54 61
17140160 2006
The pathophysiology of coronary artery aneurysms in Kawasaki disease: role of matrix metalloproteinases. 61 54
16990356 2006

Variations for Kawasaki Disease

ClinVar genetic disease variations for Kawasaki Disease:

# Gene Name Type Significance ClinVarId dbSNP ID Position
1 ITPKC NM_025194.3(ITPKC):c.1155+9G>C SNV risk factor 4277 rs28493229 GRCh37: 19:41224204-41224204
GRCh38: 19:40718299-40718299

Expression for Kawasaki Disease

Search GEO for disease gene expression data for Kawasaki Disease.

Pathways for Kawasaki Disease

Pathways related to Kawasaki Disease according to GeneCards Suite gene sharing:

(show all 34)
# Super pathways Score Top Affiliating Genes
Show member pathways
13.98 TNF TIMP1 SELL S100A12 MYD88 MMP9
Show member pathways
Show member pathways
Show member pathways
12.32 TNF MMP9 MMP3 IL6 CCL2
Show member pathways
12.28 TNF IL6 CRP CCL2
6 12.22 MMP9 MMP3 IL6 ELANE
7 12.21 TNF MYD88 IL6 HSPD1 FCGR2A
8 12.19 TNF MYD88 IL6 CCL2
9 12.07 TNF SELE MMP9 CCL2
10 12.06 TNF MYD88 IL6 FCGR2A CCL2
11 11.98 TNF SELL IL6 FCGR2A
12 11.97 TNF MMP9 IL6 FCGR2A CCL2
13 11.91 TNF SELE IL6 CCL2
14 11.87 TNF MMP3 IL6 CCL2
15 11.84 TIMP1 MMP9 MMP3 CCL2
Show member pathways
Show member pathways
11.77 TIMP1 IL6 CRP
18 11.71 TNF MYD88 IL6
19 11.7 TNF SELL IL6
20 11.69 TNF TIMP1 MMP9 MMP3 IL6 CCL2
21 11.64 TNF IL6 FCGR2A
22 11.6 TIMP1 MMP9 MMP3
23 11.6 TNF MYD88 IL6 HSPD1
Show member pathways
25 11.53 TNF TIMP1 IL6 CCL2
26 11.52 TNF TIMP1 MMP9 IL6 CCL2
27 11.49 TNF MMP9 IL6 ELANE
28 11.48 TNF SELE MMP9 MMP3 IL6 CCL2
29 11.35 TNF SELE MYD88 IL6
30 11.35 TNF SELE MYD88 IL6 CCL2
31 11.32 MMP9 IL6 CCL2
32 11.1 TIMP1 MYD88 MMP9 CCL2
33 11.02 TNF IL6 CRP

GO Terms for Kawasaki Disease

Cellular components related to Kawasaki Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.7 TNF TIMP1 S100A12 NPPB MMP9 MMP3
2 extracellular space GO:0005615 9.47 TNF TIMP1 SELE S100A12 NPPB MMP9

Biological processes related to Kawasaki Disease according to GeneCards Suite gene sharing:

(show all 31)
# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 10.04 SELL S100A12 MMP9 FCGR2A ELANE
2 defense response to bacterium GO:0042742 9.93 TNF S100A12 MYD88 MBL2 ELANE
3 response to lipopolysaccharide GO:0032496 9.91 SELE MYD88 HSPD1 ELANE
4 cellular response to lipopolysaccharide GO:0071222 9.9 TNF MYD88 IL6 CCL2
5 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.89 TNF S100A12 MYD88 IL6
6 leukocyte migration GO:0050900 9.88 TNF SELL SELE MMP9 ELANE
7 response to glucocorticoid GO:0051384 9.82 TNF IL6 HSPD1
8 positive regulation of interleukin-6 production GO:0032755 9.81 TNF MYD88 IL6 HSPD1
9 positive regulation of MAP kinase activity GO:0043406 9.79 TNF S100A12 ELANE
10 humoral immune response GO:0006959 9.79 TNF IL6 CCL2
11 monocyte chemotaxis GO:0002548 9.76 S100A12 IL6 CCL2
12 extracellular matrix disassembly GO:0022617 9.76 TIMP1 MMP9 MMP3 ELANE
13 positive regulation of chemokine production GO:0032722 9.75 TNF MYD88 IL6
14 acute-phase response GO:0006953 9.74 MBL2 IL6 CRP
15 positive regulation of interleukin-10 production GO:0032733 9.73 MIR145 IL6 HSPD1
16 positive regulation of smooth muscle cell proliferation GO:0048661 9.73 TNF MYD88 IL6 ELANE
17 lipopolysaccharide-mediated signaling pathway GO:0031663 9.72 TNF MYD88 CCL2
18 positive regulation of interleukin-8 production GO:0032757 9.71 TNF MYD88 IL6 ELANE
19 positive regulation of cytokine production involved in inflammatory response GO:1900017 9.7 TNF MYD88 IL6
20 leukocyte tethering or rolling GO:0050901 9.67 TNF SELL SELE
21 negative regulation of chemokine production GO:0032682 9.65 IL6 ELANE
22 positive regulation of leukocyte tethering or rolling GO:1903238 9.64 SELE ELANE
23 response to peptidoglycan GO:0032494 9.63 MYD88 IL6
24 vascular endothelial growth factor production GO:0010573 9.63 TNF IL6
25 opsonization GO:0008228 9.62 MBL2 CRP
26 defense response to Gram-positive bacterium GO:0050830 9.55 TNF MYD88 MBL2 IL6 CRP
27 regulation of neuroinflammatory response GO:0150077 9.54 MMP9 MMP3 IL6
28 cellular response to UV-A GO:0071492 9.5 TIMP1 MMP9 MMP3
29 inflammatory response GO:0006954 9.5 TNF SELE S100A12 MYD88 IL6 CRP
30 negative regulation of lipid storage GO:0010888 9.43 TNF IL6 CRP
31 cytokine-mediated signaling pathway GO:0019221 9.17 TNF TIMP1 MYD88 MMP9 MMP3 IL6

Molecular functions related to Kawasaki Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.26 TNF TIMP1 IL6 CCL2
2 protease binding GO:0002020 9.02 TNF TIMP1 SELL HSPD1 ELANE

Sources for Kawasaki Disease

9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
31 HPO
32 ICD10
33 ICD10 via Orphanet
37 LifeMap
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
69 Tocris
71 UMLS via Orphanet
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