Kawasaki Disease

  • Medical Author:
    Charles Patrick Davis, MD, PhD

    Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.

  • Medical Editor: John P. Cunha, DO, FACOEP
    John P. Cunha, DO, FACOEP

    John P. Cunha, DO, FACOEP

    John P. Cunha, DO, is a U.S. board-certified Emergency Medicine Physician. Dr. Cunha's educational background includes a BS in Biology from Rutgers, the State University of New Jersey, and a DO from the Kansas City University of Medicine and Biosciences in Kansas City, MO. He completed residency training in Emergency Medicine at Newark Beth Israel Medical Center in Newark, New Jersey.

Symptoms of Rheumatoid Arthritis

Kawasaki disease facts

  • 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.

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

Kawasaki disease is an uncommon illness in children that is characterized by high fever of at least five days' duration together with at least four of the following five findings:

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

The above criteria are used to make a diagnosis of Kawasaki disease. The terminology "incomplete Kawasaki disease" is sometimes used for patients who are diagnosed with only some features of classical Kawasaki disease. Most patients 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.

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Kawasaki Disease Symptoms and Signs

High fever is a prominent symptom in children with Kawasaki disease. The fever is persistent and lasts for at least five days. Nonspecific symptoms can precede or accompany the fever, including

  • fussiness,
  • nausea,
  • vomiting,
  • runny nose,
  • cough,
  • diarrhea,
  • joint pain,
  • abdominal pain,
  • and decreased intake of food and liquids.

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 and risk factors of Kawasaki disease?

The cause is not known. Microorganisms and toxins have been suspected, but none has been identified to date. It is common for Kawasaki disease to occur after a preceding infection, such as tonsillitis, ear infection, pneumonia, urine infection, or gastrointestinal infection. Genetic factors (genetic variants in some patients) and the immune system seem to play roles in the disease, which is characterized by immune activation. The disease is more common in those of Japanese or Korean descent.

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 usual 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 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 inflammation of the lining of the heart (pericarditis), joints (arthritis), or covering of the brain and spinal cord (meningitis), and the other heart problems listed below in the section on serious complications. The outlook (prognosis) for an individual affected is primarily determined by the seriousness of any heart complications.

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.

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 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. Children are typically evaluated with an electrocardiogram (EKG) and an ultrasound test of the heart (echocardiogram). Monitoring for this problem is crucial throughout life as it can develop as a late aftereffect of Kawasaki disease. Other arteries that can become inflamed include the arteries of the lungs, neck, and abdomen. These effects can lead to breathing problems, headaches, and abdominal pain, respectively. Blood tests to measure the degree of inflammation (such as C-reactive protein, or CRP, and erythrocyte sedimentation rate, or ESR) are often used to monitor the activity of the disease.

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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. 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).

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.

However, the outcome is not so favorable in every child. 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.

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>.

Bradley, D.J., and M.P. Glode. "Kawasaki Disease. The Mystery Continues." West J Med. 168.1 January 1998: 23-29. <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1304746/>.

Fulton, D.R., and J.W. Newburger. "Long-Term Cardiac Sequelae of Kawasaki Disease." Curr Rheumatol Rep 2.4 August 2000: 324-329. <https://www.ncbi.nlm.nih.gov/pubmed/11123078>.

Kato, S., et al. "HLA Antigens in Kawasaki Disease." Pediatrics 61.2 February 1978: 252-255. <https://www.ncbi.nlm.nih.gov/pubmed/634680>.

Klippel, J.H., et al. Primer on the Rheumatic Diseases. New York: Springer, 2008.

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/>.

Quick GuideChildhood Diseases: Measles, Mumps, & More

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Reviewed on 11/8/2017
References
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>.

Bradley, D.J., and M.P. Glode. "Kawasaki Disease. The Mystery Continues." West J Med. 168.1 January 1998: 23-29. <https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1304746/>.

Fulton, D.R., and J.W. Newburger. "Long-Term Cardiac Sequelae of Kawasaki Disease." Curr Rheumatol Rep 2.4 August 2000: 324-329. <https://www.ncbi.nlm.nih.gov/pubmed/11123078>.

Kato, S., et al. "HLA Antigens in Kawasaki Disease." Pediatrics 61.2 February 1978: 252-255. <https://www.ncbi.nlm.nih.gov/pubmed/634680>.

Klippel, J.H., et al. Primer on the Rheumatic Diseases. New York: Springer, 2008.

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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