- Kawasaki Syndrome
- Kawasaki Disease
- Kawasaki disease
- What is Kawasaki disease?
- Who gets Kawasaki disease?
- What causes Kawasaki disease?
- What are the signs and symptoms of Kawasaki disease?
- How is Kawasaki disease diagnosed?
- What is the treatment of Kawasaki disease?
- What are the complications of Kawasaki disease?
- What other information do parents of children treated for Kawasaki disease need to know?
- Adult-onset Kawasaki disease (mucocutaneous lymph node syndrome) and concurrent Coxsackievirus A4 infection: a case report
- Kawasaki Disease in Children
- What is Kawasaki disease in children?
- Which children are at risk for Kawasaki disease?
- What causes Kawasaki disease in a child?
- What are the symptoms of Kawasaki disease in a child?
- How is Kawasaki disease diagnosed in a child?
- How is Kawasaki disease treated in a child?
- What are possible complications of Kawasaki disease in a child?
- How is Kawasaki disease managed in a child?
- When should I call my child’s healthcare provider?
- Key points about Kawasaki disease in children
- Next steps
- What is Kawasaki disease?
- How often does it occur?
- Who is affected by Kawasaki disease?
- What causes Kawasaki disease?
- Why is Kawasaki disease a concern?
- What are the symptoms of Kawasaki disease?
- How is Kawasaki disease diagnosed?
- Diagnostic tests may also include:
- Treatment for Kawasaki disease:
- Can Kawasaki disease be prevented?
- Long-term outlook after having Kawasaki disease:
Published: January, 2019
Kawasaki disease is a rare illness that typically strikes children younger than age 5. It is also known as mucocutaneous lymph node disease. Kawasaki disease is a mysterious illness of unknown cause, although some scientists suspect that the cause may be an infection (such as a virus or a toxin from a bacterium.
Kawasaki disease was first identified among Japanese children in 1967. Within nine years, the illness had been reported in American children living in Hawaii. Although researchers assume that the Kawasaki disease could have been caused by an infection that was carried between Japan and Hawaii, this has never been confirmed. Recent evidence suggests that inherited (genetic) factors may also be important. But the cause of Kawasaki disease remains a mystery.
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What is Kawasaki disease?
Kawasaki disease is an acute febrile illness with inflammation of small- and medium-sized blood vessels throughout the body, in particular, the coronary arteries (blood vessels around the heart).
Kawasaki disease was previously known as mucocutaneous lymph node syndrome. It was first described in Japan in 1967 by Dr Tomisaku Kawasaki, a paediatrician.
Without treatment Kawasaki disease is usually self-limiting illness and resolves spontaneously within 4-8 weeks. However, about 20% of untreated cases develop coronary artery damage and approximately 2% of patients will die, most commonly from a heart attack. This outlook is improved significantly with appropriate treatment.
Who gets Kawasaki disease?
Eighty percent of cases occur in children younger than 5 years of age with a peak incidence between 1 to 2 years. The disease is very uncommon in those over 14 years old and in adults. Overall it occurs more commonly in boys than girls.
Although cases of Kawasaki disease have been reported in children of all ethnic origins, the highest incidence is in children of Asian descent especially Japanese. There are 5000–6000 cases each year in Japan.
What causes Kawasaki disease?
The cause of Kawasaki disease is unknown.
What are the signs and symptoms of Kawasaki disease?
There are several clinically apparent stages of Kawasaki disease.
Typically a child with Kawasaki disease has a high swinging fever (beyond 39C), associated with a number of other features. The 5 cardinal signs of Kawasaki disease are:
- Rash – the rash of Kawasaki disease may be morbilliform (measles-like), maculopapular (red patches and bumps), erythematous (red skin) or target-like and may be persistent over days or evanescent. Skin peeling may occur in the convalescent stage of the illness.
- Oral signs – the typical changes include redness within the mouth or on the pharynx, strawberry tongue, and red or cracked lips.
- Eye signs – redness of the bulbar conjunctivae (whites of the eyes) without exudate or stickiness.
- Peripheral limb signs – including firm swelling of the hands and feet, sometimes including the fingers and toes, with redness of the palms and soles. Periungual desquamation (peeling of skin around the fingernails) may occur during the convalescent stage of the illness.
- Lymphadenopathy – swollen lymph glands can occur, often on one side of the neck. One lymph gland of at least 1.5cm in length is considered diagnostically enlarged.
Not all of the cardinal signs may be present in any one child with Kawasaki disease and not all features may be present at the same time. Some features may appear and disappear before others arise.
Children with Kawasaki disease are often unusually irritable, out of proportion to the other signs exhibited. They may also have a range of other non-specific symptoms and signs including abdominal pain, diarrhoea, dysuria (pain passing urine), joint pain or arthritis, signs of meningitis or heart failure.
How is Kawasaki disease diagnosed?
There is no specific lab test that establishes the diagnosis of Kawasaki disease. definitively. The diagnosis is considered established when the following diagnostic criteria are met:
- Fever for at least 5 days AND
- At least 4 of the 5 cardinal signs listed above AND
- The absence of any other illness to account for the signs and symptoms.
Atypical or incomplete cases of Kawasaki disease, in which patients have fever and fewer than 4 of the 5 cardinal features, are now diagnosed more commonly. In these children, the diagnosis may be supported by findings on an early 2-dimensional echocardiography (heart ultrasound) to detect coronary artery disease or other signs of acute heart disease.
Illnesses that might present with some of the features of Kawasaki disease include scarlet fever, staphylococcal scalded skin syndrome, measles and other viral exanthems (viral rashes), systemic onset juvenile arthritis (juvenile rheumatoid arthritis or Stills disease) and drug reactions (morbilliform eruption, drug hypersensitivity syndrome and Stevens Johnson syndrome / toxic epidermal necrolysis)
What is the treatment of Kawasaki disease?
Usually children are treated with antipyretic and analgesic medication (eg paracetamol / acetominophen) until the 5th day of fever is reached. Once the diagnosis of Kawasaki disease is made a single large dose of intravenous immunoglobulin (IVIG), which is purified antibodies collected from many blood donations, is given. IVIG is most effective when given between the 5th and 10th days of illness. Low dose oral aspirin is also usually commenced at this time.
IVIG greatly reduces the risk of coronary artery disease and low dose aspirin reduces the risk of clotting within the coronary artery should coronary artery disease develop. Once children are treated they generally improve rapidly; most of the acute symptoms and signs resolve within 24 to 48 hours, and the fever abates. If the signs and symptoms fail to resolve or recur within days then a 2nd dose of IVIG is given.
What are the complications of Kawasaki disease?
The main complications of Kawasaki disease are the development of dilatation and/or narrowing of one or more coronary artery. These can lead to angina, myocardial infarction or sudden death. When children are treated with IVIG up to 10 days into the illness and respond quickly, the risk of subsequent coronary artery abnormality is reduced to 2-4% and most of these have mild abnormalities many of which resolve.
Follow-up echocardiography is performed on all children with Kawasaki disease at about 6 to 8 weeks to look for coronary artery abnormalities and to check the heart is otherwise normal. If coronary artery abnormalities are demonstrated then ongoing anticoagulation treatment, such as daily aspirin, will be needed and further heart imaging will be required.
What other information do parents of children treated for Kawasaki disease need to know?
- Kawasaki disease cannot be passed on to other family members.
- There is a 2% risk of recurrence of Kawasaki disease within a period of several years.
- IVIG affects the efficacy of live virus vaccines, i.e. MMR (measles, mumps, rubella) and varicella (chickenpox) vaccines, and these should be delayed for 11 months after the last dose of IVIG is given or repeated after 11 months if given earlier.
- There is evidence that children and young people who have echocardiographic evidence of coronary artery aneurysm are more likely to develop coronary artery disease in adulthood than the general population and at an earlier age. Those with significant coronary artery disease should be followed long term by a cardiologist. Regular health examinations and cardiovascular risk assessments for these children throughout adulthood is advised.
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What Is Kawasaki Disease?
Kawasaki disease is an illness that causes
(swelling and redness) in blood vessels throughout the body. It happens in three phases, and a lasting fever usually is the first sign.
The condition most often affects kids younger than 5 years old. When symptoms are noticed early and treated, kids with Kawasaki disease begin to feel better within a few days.
What Are the Signs & Symptoms of Kawasaki Disease?
Kawasaki disease has telltale symptoms and signs that appear in phases. The first phase, which can last for up to 2 weeks, usually involves a fever that lasts for at least 5 days.
Other symptoms include:
- red (“bloodshot”) eyes
- a pink rash on the back, belly, arms, legs, and genital area
- red, dry, cracked lips
- a “strawberry” tongue (white coating with red bumps on the tongue)
- a sore throat
- swollen palms of the hands and soles of the feet with a purple-red color
- swollen lymph glands in the neck
The second phase usually begins 2 weeks after the fever started. Symptoms can include:
- peeling skin on the hands and feet
- joint pain
- belly pain
What Problems Can Happen?
Doctors can treat the symptoms of Kawasaki disease when it’s caught early. Most kids will feel better within a few days of starting treatment.
If the condition isn’t found until later, patients can have serious complications that affect the heart, such as:
- an aneurysm (a bulge in the wall) of the coronary arteries, which supply blood to the heart
- inflammation of the heart muscle, lining, valves, and the outer membrane around the heart
- arrhythmias, which are changes in the normal pattern of the heartbeat
- problems with some heart valves
What Causes Kawasaki Disease?
Doctors don’t know what causes Kawasaki disease. They believe it doesn’t spread from person to person. It’s most common among children of Japanese and Korean descent, but can affect any child.
How Is Kawasaki Disease Diagnosed?
Kawasaki disease symptoms can look similar to those of other childhood viral and bacterial illnesses. Doctors usually diagnose it by asking about the symptoms (such as a long-lasting fever) and doing an exam.
If Kawasaki disease looks likely, the doctor:
- will order tests to check the heart, such as an echocardiogram
- might test blood and urine (pee) samples to rule out other conditions, such as scarlet fever, measles, Rocky Mountain spotted fever, or juvenile rheumatoid arthritis
How Is Kawasaki Disease Treated?
Doctors usually treat kids with Kawasaki disease by giving them:
- intravenous (IV) dose of immune globulin (IVIG): These antibodies (proteins) help fight infections. IVIG treatment also lowers the risk of coronary artery aneurysms. IVIG is given once.
- high-dose aspirin given by mouth to treat inflammation. Patients take aspirin until blood tests show that the inflammation has improved.
Treatment begins as soon as possible. In some children, IVIG may not work and doctors give steroids instead. Steroids can help prevent coronary aneurysms.
It’s very important for children on high-dose aspirin to get the annual flu vaccine to help prevent this viral illness. That’s because there’s a small risk of a rare condition called Reye syndrome in children who take aspirin during a viral illness.
Most children with Kawasaki disease start to get much better after a single treatment with immune globulin, though sometimes more doses are needed.
What Else Should I Know?
Most kids with Kawasaki disease recover completely, especially when they are diagnosed and treated early. Some, especially those who develop heart problems from Kawasaki disease, might need more testing and to see a
(a doctor who specializes in conditions that affect the heart). Reviewed by: Kate M. Cronan, MD Date reviewed: July 2019
Adult-onset Kawasaki disease (mucocutaneous lymph node syndrome) and concurrent Coxsackievirus A4 infection: a case report
Kawasaki disease (KD) or mucocutaneous lymph node syndrome is a childhood disorder that was first reported by Tomisaku Kawasaki in 1967.1 The characteristic signs are conjunctival congestion, skin rash, skin desquamation of the peripheral limbs, erythema of the oral cavity, lips, and palms, and cervical lymphadenopathy.1 KD most commonly develops in infants. Approximately 85% of the patients with KD are under 5 years of age.2,3 The occurrence of KD is rare in adolescents and adults.4 The specific cause of KD is still unclear, but some reports suggest that it is triggered by bacterial or viral infections.5–7 Herein, we describe a rare case of adult-onset KD which revealed to be concurrently infected by Coxsackievirus A4.
The patient consented to his case being included in this case report. Institutional Review Board approval was not deemed necessary for the following retrospective case report. All principles outlined in the Declaration of Helsinki were followed. A 37-year-old, previously healthy Japanese man was admitted to our hospital 12 days after he developed a fever of 38°C and erythema of the hands, arms, and legs. Concurrently, he developed swollen and painful fingers. Two days later, he consulted the outpatient department because of fever. He was treated with 300 mg/day of cefcapene-pivoxil and 60 mg/day of loxoprofen, but his condition did not improve. Seven days before admission to our hospital, he was admitted to another hospital for examination of the cause of the fever and erythema in the neck, abdomen, back, and legs. The laboratory test results in the previous hospital included total bilirubin of 3.58 mg/dL, aspartate aminotransferase of 48 U/L, alanine aminotransferase of 61 U/L, γ-glutamyltranspeptidase of 116 U/L, blood nitrogen urea of 45 mg/dL, and creatinine of 3.45 mg/dL, indicating liver and kidney dysfunction. On the 2nd day in the previous hospital, he developed conjunctival congestion. On day 5, desquamation of the hands, fingers, and toes was noted. He was eventually referred to our hospital for close examination and treatment.
At the time of admission to our hospital, the patient was clearly conscious. His temperature was 37.7°C, blood pressure was 136/84 mmHg, pulse rate was regular at 88 beats per minute, and respiratory rate was 28 breaths per minute. Physical examination showed desquamation around the lips (Figure 1A), fingers (Figure 1B), and feet (Figure 1C). Edematous erythema was noted on his trunk, extremities, axillae, flank, and hip. There was also brawny edema of the hands and feet and marked congestion of the conjunctivae (Figure 1D). His lips were reddish, dry, and swollen. His palatine tonsils were also slightly red. The cervical lymph nodes were not enlarged. The chest was clear to auscultation. The abdomen was soft and non-tender. No arthritis was noted, and neurological examination also yielded normal results. Laboratory data on admission revealed leukocytosis, neutrophilia, and thrombocytosis, and serum aspartate aminotransferase, alanine aminotransferase, creatinine, and C-reactive protein levels were elevated (Table 1). Repeat blood culture was sterile. Rapid test for Streptococcus pyogenes antigen was negative. The presence of antibodies to Epstein–Barr virus and cytomegalovirus indicated past infections (Table 2). Chest radiography showed no abnormalities. Computed tomography of the chest and abdomen revealed hepatic and splenic enlargement and fatty liver. A skin biopsy of the erythema on the left forearm showed lymphocyte infiltrations around vessels in the superficial layer of the epidermis. There were no findings suspected for vasculitis or drug allergy. The patient had fever for >5 days, and four additional principal signs indicative of KD based on the diagnostic criteria defined by the Centers for Disease Control and Prevention,8 namely exanthema, change in peripheral extremities, bilateral non-exudative conjunctival injection, and changes in the oropharynx, on the basis of which he was clinically diagnosed with KD. On the day of admission, he was treated with 2,700 mg/day of oral aspirin (30 mg/kg/day). On day 4, the dose of aspirin was reduced to 450 mg/day (5 mg/kg/day) because of defervescence; however, on day 5, the patient developed liver dysfunction as an adverse effect of aspirin. After day 6 in our hospital, the myalgia, congested conjunctivae, erythema, and desquamations were found to be gradually resolving. By day 13, the erythema and desquamation were completely resolved. However, the treatment was switched from aspirin to 200 mg/day of cilostazol because the alanine aminotransferase levels increased to 150 U/L (Figure 2). Subsequently, the liver function normalized, and the patient was discharged on the 13th hospital day. During hospitalization, transthoracic echocardiography disclosed no coronary aneurysms. At follow-up, coronary computed tomography performed 2 months after the onset of the disease revealed no coronary aneurysms (Figure 3).
Table 1 Laboratory data at the time of admission to our hospital (day 13 of illness)
Table 2 Microbiological data
Figure 3 Coronary computed tomography.
Note: The coronary computed tomography that was performed 2 months after the onset of the disease revealed no coronary aneurysms.
Abbreviations: LCX, left circumflex artery; LAD, left anterior descending coronary artery; RCA, right coronary artery.
Assuming that a viral infection was associated with KD in this patient, we examined the antibody titers in response to viruses that cause acute eruptive diseases. The virus neutralization test was used to determine antibody titers for Coxsackievirus, adenovirus, and echovirus in the serum, on days 5, 13, and 32 from onset of the disease. Antibody titer of Coxsackievirus A4 on day 13 was fourfold higher than that on day 5 and decreased on day 32 in the following sequence: 32-fold on day 5, 128-fold on day 13, and 32-fold on day 32 (Table 3). These results strongly suggest that the Coxsackievirus infection was present when KD occurred.
Table 3 Viral antibody titers
Notes: The bold font indicates the antibody titer of only Coxsackievirus A4 showed significant change. The antibody titer of only Coxsackievirus A4 was increased fourfold on day 13 as compared to day 5 and was decreased on day 32.
Abbreviation: NT, neutralization.
Here, we describe a case of adult-onset KD which revealed to be concurrently infected by Coxsackievirus A4. Adult-onset KD is rare, and this is a very rare case of KD and concurrent Coxsackievirus A4 infection.
KD most commonly develops in infants. The annual incidence is 67 cases per 100,000 children in Japan and 5.6 cases per 100,000 children in the USA. Children under 5 years of age constitute 88.5% of reported cases.2,3 KD occurs predominantly in children while rarely in adolescents and adults.4 The oldest reported case was that of a 68-year-old Caucasian man from France in 2005.9 The diagnostic criteria for KD as defined by the Centers for Disease Control and Prevention include unexplained fever lasting 5 days or more and at least four out of the five following criteria: 1) polymorphic exanthema; 2) changes in the peripheral extremities, that is, erythema and/or indurative edema of the palms and soles (acute phase) or desquamation around the finger tips (convalescent phase); 3) bilateral non-exudative conjunctival injection; 4) changes in the oropharynx, that is, injected or fissured lips, strawberry tongue, and injected pharynx; and 5) acute nonsuppurative cervical lymphadenopathy (>1.5 cm in diameter). Patients with fewer than four of these clinical signs can be diagnosed as having atypical KD if coronary artery abnormalities are present.8 Additionally, it is necessary to exclude the possibility of other diseases that cause fever and rash (eg, toxic shock syndrome, streptococcal scarlet fever, measles, other viral infections, Stevens–Johnson syndrome, or a drug reaction).
Desquamation is typically found during the convalescent phase. However, in patients who develop KD in adulthood, the time of onset of desquamation is varied. Some of these patients have been reported to develop desquamation during the acute phase of KD with fever.9,10 This may be associated with the fact that multiple factors cause KD and that the mechanism of KD differs between adults and children. In our patient, desquamation appeared relatively early and then improved. Although the side effects of cefcapene-pivoxil and loxoprofen should be considered in the differential diagnosis, both drugs were used without adverse effects in this patient before this episode. Item (2) in the diagnostic criteria was satisfied even if desquamation was excluded. Therefore, desquamation does not affect the diagnosis.
The etiology of KD remains unclear, although the clinical features suggest that a viral or bacterial infection may be a trigger.11 KD has features similar to those of other childhood infectious diseases. For example, similar to measles and adenovirus infection, KD has the clinical features of fever and rash. The epidemiological features including age distribution, seasonal prevalence, and occurrence of community outbreaks with geographical spread also suggest a transmissible childhood disease.12 However, an infectious agent has not yet been identified. Adenovirus, herpesvirus, Epstein–Barr virus, human coronavirus New Haven, measles virus, rotavirus, dengue virus, and retrovirus are putative etiological agents of KD. These are commonly found pathogens.13 However, the reason why only some patients infected with these viruses develop KD is unknown. A report demonstrated coinfection with Coxsackievirus B3 in a patient with KD.13
In our patient, we examined the antibody titers of viruses that cause an acute eruptive disease, such as Coxsackievirus (types A2–A5, B1–B4), adenovirus (types 2–4), echovirus (types 1, 3, 5, 7), herpes simplex virus, Epstein–Barr virus, cytomegalovirus, human immunodeficiency virus, and human parvovirus. We analyzed serum samples taken on days 5, 13, and 32 of illness for antibody titers of Coxsackievirus, adenovirus, and echovirus. The day 5 serum sample was taken from the previous hospital. The antibody titer of only Coxsackievirus A4 was increased fourfold on day 13 as compared to day 5 and was decreased on day 32. This strongly suggests that the Coxsackievirus infection was present when KD occurred. Coxsackievirus A4 is a member of the Picornaviridae family of viruses in the Enterovirus genus. It can be a cause of herpangina and myocarditis. Rigante et al reported the cases of Kawasaki syndrome and concurrent Coxsackievirus B3 infection.13 However, to the best of our knowledge, there have been no reports of KD cases and concurrent Coxsackievirus A4 infection thus far. There are various types of adenovirus and Coxsackievirus, and tests for proving these common viral infections (eg, serological antibody, polymerase chain reaction) are performed in few cases. Therefore, such coinfections remain undiagnosed and are thus likely unreported.
It is important not only to manage symptoms in the acute phase of KD but also to prevent the cardiovascular after-effects. There are three essential treatments for KD: 1) intravenous immunoglobulin (IVIG) to obtain an anti-inflammatory effect, 2) aspirin for anti-inflammatory and antiplatelet effects, and 3) management of any complications (eg, meningitis or disseminated intravascular coagulation). IVIG can mitigate inflammation and the coronary artery complications. A dose of 2 g/kg of IVIG in a single infusion has been found to be effective. This therapy should be initiated within the first 10 days of illness.14,15 IVIG has not been demonstrated to be effective if administered after the first 10 days of illness. Furthermore, one report has noted a risk of unfavorable effects with IVIG regarding cardiac sequelae if IVIG is started on day 9 or later.16 In our patient, IVIG was not administered because he was admitted to our hospital on the 13th day of illness.
Aspirin is often administered with IVIG in patients with KD. In the acute phase, the American Heart Association recommends high-dose aspirin (80–100 mg/kg/day) to achieve an anti-inflammatory effect.14 However, it is not clear if a higher dose would be more effective than the lower dose (30–50 mg/kg/day).17 When the patient remains afebrile for 48–72 hours, the aspirin dose is lowered (3–5 mg/kg/day) and maintained to achieve an antiplatelet effect until the patient shows no evidence of coronary changes for 6–8 weeks after onset.14 In addition, regular echocardiographic evaluation should be performed to assess for the possibility of coronary artery aneurysm.
In our patient, 30 mg/kg/day of aspirin was used to achieve an anti-inflammatory effect from day 13 to day 15 of illness to ensure better tolerance to gastrointestinal and other side effects. The dose of aspirin was reduced to 5 mg/kg/day on day 16 of illness. Because the patient developed liver dysfunction as an adverse effect of aspirin on day 5, the treatment was switched to 200 mg/day of cilostazol on day 13.
It is still unclear if the treatment for KD in adults should be the same as in infants, especially with regard to the dose and duration of IVIG and aspirin administration.
This rare case of adult-onset KD suggests that the disease can be concurrent Coxsackievirus A4 infection. Although KD is an acute childhood disease, with fever as the main sign, it should be considered in the differential diagnosis also when adult patients present with unexplained fever and a rash.
Yuki Ueda contributed to manuscript editing. Tsuneaki Kenzaka contributed to management of the case, and manuscript editing and correction. Ayako Noda and Yu Yamamoto contributed to clinical management of the case and revision of the manuscript. Masami Matsumura contributed to manuscript correction and editing of the captions for the illustrations. All the authors read and approved the final version of the manuscript. All authors contributed toward data analysis, drafting and critically revising the paper and agree to be accountable for all aspects of the work.
The authors declare that they have no conflicts of interest related to this work.
Kawasaki T. Acute febrile muco-cutaneous lymph node syndrome in young children with unique digital desquamation. Jpn J Allergol. 1967;16:178–222.
Yanagawa H, Kawasaki T, Shigematsu I. Nationwide survey on Kawasaki disease in Japan. Pediatrics. 1987;80:58–62.
Rauch AM. Kawasaki syndrome: critical review of US epidemiology. Prog Clin Biol Res. 1987;250:33–40.
Jackson JL, Kunkel MR, Libow L, Gates RH. Adult Kawasaki disease. Report of two cases treated with intravenous gamma globulin. Arch Intern Med. 1994;154:1398–1405.
Hall M, Hoyt L, Ferrieri P, Schlievert PM, Jenson HB. Kawasaki syndrome-like illness associated with infection caused by enterotoxin B-secreting Staphylococcus aureus. Clin Infect Dis. 1999;29:586–589.
Matsubara K, Fukaya T, Miwa K, et al. Development of serum IgM antibodies against super antigens of Staphylococcus aureus and Streptococcus pyogenes in Kawasaki disease. Clin Exp Immunol. 2006;143:427–434.
Esper F, Shapiro ED, Weibel C, Ferguson D, Landry ML, Kahn JS. Association between a novel human coronavirus and Kawasaki disease. J Infect Dis. 2005;191:499–502.
Pascal S, Katia S, Amar S, Denis VD, Guillaume M, Christiane B. Adult Kawasaki disease: report of two cases and literature review. Semin Arthritis Rheum. 2005;34:785–792.
Rauch AM, Hurwitz ES. Center for Disease Control (CDC) case definition for Kawasaki syndrome. Pediatr Infect Dis. 1985;4:702–703.
Gomard-Mennesson E, Landron C, Dauphin C, et al. Kawasaki disease in adults: report of 10 cases. Medicine (Baltimore). 2010;89(3):149–158.
Principi N, Rigante D, Esposito S. The role of infection in Kawasaki syndrome. J Infect. 2013;67:1–10.
Burns JC, Cayan DR, Tong G, et al. Seasonality and temporal clustering of Kawasaki syndrome. Epidemiology. 2005;16:220–225.
Rigante D, Cantarini L, Piastra M, et al. Kawasaki syndrome and concurrent Coxsackie virus B3 infection. Rheumatol Int. 2012;32:4037–4040.
Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics. 2004;114:1708–1733.
Dajani AS, Taubert KA, Gerber MA, et al. Diagnosis and therapy of Kawasaki disease in children. Circulation. 1993;87:1776–1780.
Yanagawa H, Nakamura Y, Sakata K, Yashiro M. Use of intravenous γ-globulin for Kawasaki disease: effects on cardiac sequelae. Pediatr Cardiol. 1997;18:19–23.
Brogan PA, Bose A, Burgner D, et al. Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research. Arch Dis Child. 2002;86:286–290.
Kawasaki Disease in Children
What is Kawasaki disease in children?
Kawasaki disease is a rare illness that most commonly affects children ages 0 to 5, but can sometimes affect children up to the age of 13. It is a type of vasculitis. Vasculitis means inflammation of the blood vessels. It can affect the whole body, including the blood vessels of the heart (coronary arteries). The cause of Kawasaki disease is unknown. Without treatment, affected children are at higher risk of developing problems with the coronary arteries. Other areas of the heart may also be affected. With timely treatment, most children recover with no lasting problems.
Which children are at risk for Kawasaki disease?
Children of any race or ethnic group can get Kawasaki disease. It’s more common in children whose families are from East Asia or Asian ancestry. Most children who get Kawasaki disease are younger than 5 years old. It occurs in boys more often than in girls.
What causes Kawasaki disease in a child?
The cause of Kawasaki disease is not known. Researchers think it may be the result of an infection.
What are the symptoms of Kawasaki disease in a child?
These are common symptoms of Kawasaki disease:
Fever of 102.0° F to 104.0° F (38.8°C to 40.0°C) that lasts for at least 5 days
A swollen lymph node, usually in the neck
Swollen hands and feet
Red and dry cracked lips
Red tongue with white spots (called “strawberry tongue”)
Fast heart rate
Diarrhea or vomiting
The symptoms of Kawasaki disease can look like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.
How is Kawasaki disease diagnosed in a child?
Your child’s healthcare provider can usually diagnose Kawasaki disease by his or her symptoms and physical exam.
To diagnose Kawasaki, other causes for the symptoms must be ruled out. A fever for 5 days must be present in addition to having 4 out of 5 of the following:
Changes in the lining of the mouth
Skin changes in the hands and feet
Swollen lymph nodes
Other recommended tests include:
Lab tests. Blood and urine samples are taken to check for signs of inflammation. These are also used to help rule out other health problems.
Electrocardiography (ECG). This test records the electrical activity of the heart through small, sticky patches on the child’s chest. The patches are connected to a machine with wires. The machine records the electrical activity. This helps check for problems with heart rhythm and heart structure.
Echocardiography (echo). This test uses sound waves to create a picture of the heart. This can show problems with heart structure, valves, and heart function.
Cardiac catheterization. This test uses a small tube that goes into the blood vessels and takes pictures of the coronary arteries using contrast and X-ray.
How is Kawasaki disease treated in a child?
Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. Treatment typically starts as soon as the problem is suspected. Your child may need to stay in the hospital for a few days or longer.
Your child’s healthcare provider may prescribe aspirin or IV (intravenous) gamma globulin (IVIG). Corticosteroids and other medicines may also be prescribed if aspirin and IVIG don’t work well. Once your child is home, he or she may need to take low-dose aspirin for 6 to 8 weeks. Don’t give your child aspirin without first talking with the child’s healthcare provider. If your child develops heart problems, the provider may send you to a pediatric cardiologist. This is a doctor with special training to treat children’s heart problems. Your child may need medicine, procedures, or surgery.
What are possible complications of Kawasaki disease in a child?
Most children with Kawasaki disease get better within a few weeks. But serious complications may occur. Those involving the heart include:
Weakening of one of the heart’s arteries (coronary artery aneurysm)
Heart muscle that doesn’t work well or heart attack
Inflammation of the heart muscle (myocarditis), lining of the heart (endocarditis), or covering of the heart (pericarditis)
Heart valves that don’t work well
Kawasaki disease may also affect other body systems. This includes the nervous, immune, digestive, and urinary systems.
How is Kawasaki disease managed in a child?
If your child has a coronary artery aneurysm, he or she will need echocardiograms, sometimes for several years after the illness. Your child may need more treatment, including blood thinners to prevent clots. It’s important to keep follow-up visits with your child’s healthcare provider, even if your child is feeling well.
There is a risk for early coronary artery disease after having Kawasaki disease, including early heart attacks. Your child will need to follow a heart-healthy lifestyle for life. This includes eating healthy foods, getting regular exercise, and not smoking. Your child should have regular follow-up with a cardiologist throughout his or her life.
Talk with your child’s healthcare provider about what to expect for your child.
When should I call my child’s healthcare provider?
Call your child’s healthcare provider if your child has the symptoms of Kawasaki disease. If your child is diagnosed with Kawasaki disease, keep all follow-up appointments. Also watch for signs or symptoms of complications, including:
Poor feeding or eating
Key points about Kawasaki disease in children
Kawasaki disease is a serious condition that affects young children. It can damage blood vessels throughout the body.
Kawasaki disease is diagnosed by having certain symptoms. For example, a fever lasting at least 5 days.
Your child’s healthcare provider will treat Kawasaki with aspirin, intravenous immune globulin (IVIG), or other medicines.
A child with Kawasaki disease may have serious complications, especially ones affecting the heart.
Tips to help you get the most from a visit to your child’s healthcare provider:
Know the reason for the visit and what you want to happen.
Before your visit, write down questions you want answered.
At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
Ask if your child’s condition can be treated in other ways.
Know why a test or procedure is recommended and what the results could mean.
Know what to expect if your child does not take the medicine or have the test or procedure.
If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.
What is Kawasaki disease?
Kawasaki disease is the most common form of vasculitis that primarily affects children. The disease produces irritation and inflammation of many tissues of the body, including the hands, feet, whites of the eyes, mouth, lips, and throat. High fever and swelling of the lymph nodes in the neck are also characteristic of this illness. The inflammation is uncomfortable, but resolves with time. However, the main threat from Kawasaki disease comes from its effect on the heart and blood vessels. Heart-related complications can be temporary or may affect the child long-term. The heart, particularly the coronary arteries, is affected in as many as 20 percent of children with Kawasaki disease.
Another name for Kawasaki disease is mucocutaneous lymph node syndrome.
How often does it occur?
Kawasaki disease is fairly common in the U.S. According to the American Heart Association, the illness is a major cause of heart disease in children. About 1,800 new cases are diagnosed in the US each year, and the incidence is on the rise. Kawasaki disease, together with acute rheumatic fever, is the leading cause of acquired heart disease in children in the U.S. and Japan.
Who is affected by Kawasaki disease?
Kawasaki disease occurs more often in Japan than in any other country. In the U.S., children of Asian or Asian American heritage are affected more often than other races, although Kawasaki disease can occur in any racial or ethnic group.
The vast majority of children who develop Kawasaki disease are under age 5. The average age child seen with the illness is 2 years old. It occurs in boys twice as often as in girls.
What causes Kawasaki disease?
It is not clear what causes Kawasaki disease. Scientists believe a virus may be responsible, but current research is still underway. Kawasaki disease does not appear to be contagious, nor does it appear to be hereditary. It was once thought that Kawasaki disease was linked to recent rug or carpet cleaning; however, no studies have shown this to be a cause of the disease.
It is rare for more than one child in a family to develop the disease. As a result, less than 2 percent of persons with Kawasaki disease develop the disease more than once.
Why is Kawasaki disease a concern?
Kawasaki disease can be a very uncomfortable illness, since it causes fever, as well as irritation and inflammation in many tissues of the body. However, these symptoms usually run their course and resolve within a few weeks. The primary concern with Kawasaki disease is heart and blood vessel involvement.
The coronary arteries are the vessels that provide the heart muscle with an oxygen-rich blood supply. Kawasaki disease can weaken the wall of one or more of the coronary arteries, causing it to bulge or balloon out. This weakened, ballooned area is called an aneurysm.
Blood clots can form in the ballooned area and possibly block the blood flow through the coronary artery. When this happens, the heart muscle will no longer receive an adequate supply of oxygen-rich (red) blood, and the heart muscle can be damaged.
The illness may also cause the heart muscle (myocardium) to be irritated and inflamed, as well as the membrane covering the heart (pericardium). Irregular heart rhythms and heart valve problems may also occur with Kawasaki disease.
In most cases, the effects on the heart caused by Kawasaki disease are temporary, and resolve within five or six weeks. However, coronary artery problems may sometimes persist for longer periods of time.
What are the symptoms of Kawasaki disease?
The following are the most common symptoms of Kawasaki disease. However, each child may experience symptoms differently. Symptoms may include:
- moderate to high fever (101° F to 104° F) that rises and falls for up to three weeks
- swollen lymph glands in the neck
- rash on the back, chest, abdomen, and/or groin
- bloodshot eyes
- sensitivity to light
- swollen, coated tongue
- dry, red, cracked lips
- dark red interior surfaces of the mouth
- red, swollen palms of hands and soles of feet
- peeling skin around the nailbeds, hands, or feet
- swollen, painful joints
The symptoms of Kawasaki disease may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.
How is Kawasaki disease diagnosed?
Your child’s physician will obtain a medical history, and perform a physical examination. Several of the above mentioned symptoms need to be present for your child’s physician to consider Kawasaki’s disease as the cause for the illness.
Diagnostic tests may also include:
- electrocardiogram (ECG or EKG) – a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.
- echocardiogram (echo) – a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
- x-ray – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
- complete blood count (CBC) – a measurement of size, number, and maturity of different blood cells in a specific volume of blood. The physician will look for an elevation in the numbers of white blood cells which normally multiply in the presence of infection and may notice elevated platelet levels with Kawasaki disease as well.
- erythrocyte sedimentation rate (ESR or sed rate) – a measurement of how quickly red blood cells fall to the bottom of a test tube. When swelling and inflammation are present, the blood’s proteins clump together and become heavier than normal. Thus, when measured, they fall and settle faster at the bottom of the test tube. Generally, the faster the blood cells fall, the more severe the inflammation.
- urinalysis – testing of a urine sample for protein, red blood cells, white blood cells, or casts to indicate kidney disease associated with several rheumatic diseases.
Treatment for Kawasaki disease:
Specific treatment for Kawasaki disease will be determined by your child’s physician based on:
- your child’s age, overall health, and medical history
- extent of the disease
- your child’s tolerance for specific medications, procedures, or therapies
- expectations for the course of the disease
- your opinion or preference
Prior to the diagnosis of Kawasaki disease by your child’s physician, the goal of treatment is to provide comfort for your child. Fever reduction and increasing fluids are very helpful. Acetaminophen or ibuprofen is often used for fever.
Once the diagnosis of Kawasaki disease is made, your child’s physician may prescribe aspirin to help decrease the inflammation that the illness produces, as well as to prevent clots from forming. However, always check with your child’s physician before giving your child aspirin. Intravenous (IV) medication called gamma globulin decreases the risk of the heart being affected. Intravenous gamma globulin is most often given in the hospital.
Can Kawasaki disease be prevented?
Currently, there is no known way to prevent Kawasaki disease.
Long-term outlook after having Kawasaki disease:
About 80 percent of all children have a full recovery after Kawasaki disease resolves. If an aneurysm is detected, echocardiograms will be repeated periodically, sometimes for several years after the illness. Some heart problems may not be evident right away, so it is important to keep follow-up appointments with your child’s physician, even if your child is feeling well.