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Kawasaki Disease

Kawasaki disease facts

A non-blistering rash is one of the clinical features of Kawasaki disease.
A non-blistering rash is one of the clinical features of Kawasaki disease.
  • Kawasaki disease is a syndrome of unknown cause that mainly strikes young children.
  • Kawasaki disease symptoms and signs include
  • The disease can be treated with high doses of aspirin (salicylic acid) and gamma globulin.
  • Kawasaki disease symptoms usually resolve within a month or two, but the disease should be considered a "lifelong disease" because monitoring for late-onset heart artery changes is necessary.
  • Some children with Kawasaki disease suffer coronary artery lesions.
  • Multisystem inflammatory syndrome in children (MIS-C) is different but with similar symptoms to Kawasaki disease.

What is Kawasaki disease, and how do doctors diagnose it?

The definition of Kawasaki disease is an uncommon illness in children that is characterized by high fever of at least 5 days' duration together with at least four of the following five symptoms and signs that occur in the acute phase:

  1. Inflammation with reddening of the whites (conjunctivae) of the eyes (conjunctivitis or pinkeye) without pus
  2. Redness or swelling of the feet and/or hands, or generalized skin peeling
  3. Rash on body and/or genital area
  4. Lymph nodes swelling in the neck
  5. Cracking, inflamed lips or throat, or red "strawberry" tongue

Doctors use the above criteria to make a diagnosis of Kawasaki disease. Some investigators consider this phase 1. Phase 2 signs and symptoms may occur during the disease and may include:

  1. Skin peeling (from red palms and finger tips and reddish swollen feet)
  2. Diarrhea
  3. Vomiting
  4. Abdominal pain
  5. Joint pain

In phase 3, signs and symptoms slowly abate if there are no complications. However, the person may have irritability, tiredness, and low energy for 1-2 months.

Doctors sometimes use the terminology "incomplete Kawasaki disease" for patients who receive a diagnosis with only some features of classical Kawasaki disease.

Most patients (about 77%) are under 5 years of age with a peak incidence at 18 months of age. It is the most common acquired heart disease in children. Complications may include long-term effects of heart problems and coronary artery abnormalities like vasculitis.

What is mucocutaneous lymph node syndrome?

Mucocutaneous lymph node syndrome is the original name for Kawasaki disease. The original name was quite descriptive because the disease is characterized by the typical changes in the mucous membranes that line the lips and mouth and by the enlarged and tender lymph nodes. Kawasaki disease is also termed infantile polyarteritis.

What are causes of Kawasaki disease?

The cause is not known. Microorganisms and toxins like that of scarlet fever have been suspected, but none has been identified to date.

Who develops Kawasaki disease?

Kawasaki disease typically affects children under 5 years of age. Only rarely does it affect children over 8 years of age, in which case it is sometimes referred to as atypical Kawasaki disease.

What are the symptoms and signs of Kawasaki disease?

The usual symptoms and signs of Kawasaki disease include

The rash appears in a glove-and-sock fashion over the skin of the hands and feet. The rash becomes hard, swollen (edematous), and then peels off.

What are the unusual signs and symptoms of Kawasaki disease?

Most of the common symptoms described above will resolve without complications, even if untreated. Less common findings include

The outlook (prognosis) for an individual affected is primarily determined by the seriousness of any heart complications.

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What is the difference between Kawasaki disease and Kawasaki syndrome?

They are the same. Kawasaki disease is also referred to as Kawasaki syndrome. It was first described in the late 1960s in Japan by the renowned pediatrician Tomisaku Kawasaki.

Is Kawasaki disease contagious?

No. Kawasaki disease is not believed to be a contagious illness. However, Kawasaki disease symptoms and signs often start after the patient has had an acute infection with something else. The cause of the preceding illness may be something contagious.

How can Kawasaki disease cause serious complications?

Children with Kawasaki disease can develop inflammation of the arteries of various parts of the body.

  • This inflammation of the arteries is called vasculitis. Arteries that can be affected include the arteries that supply blood to the heart muscle (the coronary arteries).
  • Vasculitis can cause weakening of the blood vessels and lead to areas of vessel widening (aneurysms).
  • The weakening occurs because of the destruction of the elastic tissue in the walls of the blood vessels.
  • Coronary artery aneurysms occur because of such injury to the blood vessels in Kawasaki disease.

Because of the potential for heart injury and coronary artery aneurysms, special tests are performed to examine the heart.

What is the treatment for Kawasaki disease?

Children affected by Kawasaki disease are hospitalized.

  • Kawasaki disease is treated with high doses of aspirin (salicylic acid) to reduce inflammation and to mildly thin the blood to prevent blood clot formation. This is one of the few times when children may take aspirin.
  • Also used in treatment is gamma globulin administered through the vein (intravenous immunoglobulin or IVIG), together with fluids. This treatment has been shown to decrease the chance of developing coronary aneurysms in the coronary arteries, especially when used early in the illness.
  • Sometimes cortisone medications are given. Persisting joint pains are treated with anti-inflammatory drugs, such as ibuprofen (Advil) or naproxen (Aleve).
  • Plasma exchange (plasmapheresis) has been reported as effective in patients who were not responding to aspirin and gamma globulin. Plasmapheresis is a procedure whereby the patient's plasma is removed from the blood and replaced with protein-containing fluids. By taking out portions of the patient's plasma, the procedure also removes antibodies and proteins that are felt to be part of the immune reaction that is causing the inflammation of the disease.

Kawasaki disease that is not responding to the traditional cornerstones of treatment, aspirin and gamma globulin infusions, can be deadly. Treatments being investigated include doxycycline, statin drugs (Lipitor, Mevacor, others), anakinra (Kineret), and cyclosporine (Neoral).

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What is the prognosis for children with Kawasaki disease?

Kawasaki disease is typically self-limited and generally resolves on its own after four to eight weeks, and with early treatment, full recovery is usual.

Rarely, Kawasaki disease can cause death from blood clots forming in abnormal areas of widening (aneurysms) of the heart arteries (coronary arteries) and myocarditis (inflammation of heart muscle). Aneurysms of the arteries to the heart (coronary arteries) can occur early or late, even when the children are adults so follow-up is recommended for those patients diagnosed with the disease. Those children with larger aneurysms have a worse prognosis because of this risk. Such aneurysms can lead to blood clots, heart attacks (myocardial infarction), and internal bleeding. Those whose echocardiograms do not demonstrate widening of the coronary arteries throughout life do best. Those with any signs of aneurysm changes require very close monitoring. Children less than 6 months of age and those with high levels of inflammation on blood testing are at highest risk.

The earlier the diagnosis is made and treatment is begun the better the outcome.

Researchers are searching for methods of detecting which children are at risk for the development of aneurysms of the coronary arteries. Further research is under way to investigate a variety of criteria for atypical variants of Kawasaki disease that do not have classical presentations.

Is it possible to prevent Kawasaki disease?

Because the cause of Kawasaki disease has not been determined, there are no measures known that can prevent the disease. Early evaluation and treatment can decrease the chance for long-term problems and/or complications.

Kawasaki disease and the new multisystem inflammatory syndrome in children (MIS-C)

Another disease recently noted to have almost identical signs and symptoms to Kawasaki disease has been linked to the COVID-19 (coronavirus) pandemic. The new disease seems more virulent than Kawasaki disease. The CDC named the disease (as of May 14, 2020) multisystem inflammatory syndrome in children or MIS-C. The following is the case definition for MIS-C provided by the CDC:

  • An individual under 21 years of age with fever, laboratory evidence of inflammation, and evidence of clinically severe illness requiring hospitalization with multisystem (>2) organ involvement (cardiac, renal, respiratory, hematologic, gastrointestinal, dermatologic or neurological); and
  • no alternative plausible diagnoses; and
  • positive for current or recent SARS-CoV-2 infection by reverse-transcriptase polymerase chain reaction, serology, or antigen test; or COVID-19 exposure within the 4 weeks prior to the onset of symptoms.

The CDC noted the fever should be at least 100.4 F (38 C) for at least 24 hours or a subjective fever lasting 24 hours. The main differences between MIS-C and Kawasaki disease are a more rapid and serious advancement of symptoms, especially fever and multisystem organ problems like cardiac and respiratory problems along with a current or recent infection with SARS-CoV-2. Some children suffer a rapid heartbeat and may develop cardiac and/or respiratory arrest.

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Reviewed on 5/18/2020
References
American College of Rheumatology National Meeting, Boston, 2007.

American Heart Association. "Kawasaki Disease." May 8, 2017. <http://www.heart.org/HEARTORG/Conditions/More/CardiovascularConditionsofChildhood/Kawasaki-Disease_UCM_308777_Article.jsp#.WgC4m1tSxhE>.

American Heart Association. "What Is Kawasaki Disease?" 2017. <http://www.heart.org/idc/groups/heart-public/@wcm/@hcm/documents/downloadable/ucm_300320.pdf>.

Baer, A.Z., et al. "Prevalence of coronary artery lesions on the initial echocardiogram in Kawasaki syndrome." Arch Pediatr Adolesc Med 160.7 July 2006: 686-690. <https://www.ncbi.nlm.nih.gov/pubmed/16818833>.

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McCrindle, Brian W., et al. "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association." Circulation 136.19 Nov. 7, 2017. <http://circ.ahajournals.org/content/early/2017/03/29/CIR.0000000000000484>.

Melish, Marian E. "Kawasaki Syndrome." Pediatrics in Review 17.5 May 1996. <http://pedsinreview.aappublications.org/content/17/5/153>.

Onouchi, Y., et al. "ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms." Nat Genet 40.1 January 2008: 35-42. <https://www.ncbi.nlm.nih.gov/pubmed/18084290>.

Ruddy, Shaun, et al., eds. Kelley's Textbook of Rheumatology. Philadelphia: W.B. Saunders Co., 2000.

Shigematsu, I., S. Shibata, H. Tamashiro, T. Kawasaki, and S. Kusakawa. "Kawasaki Disease Continues to Increase in Japan." Pediatrics 64.3 September 1979. <http://pediatrics.aappublications.org/content/64/3/386>.

Watanabe, Toru. "Pyuria in Patients With Kawasaki Disease." World J Clin Pediatr 4.2 May 8, 2015. <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4438438/>.

Wolff, Anne E., et al. "Acute Kawasaki Disease: Not Just for Kids." J Gen Intern Med 22.5 May 2007: 681-684. <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1852903/>.
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