Kawasaki Disease

 

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Definition/Description

      Kawasaki disease, also known as mucocutaneous lymph node syndrome (pathology), is a medium-sized vessel vasculitis that affects children, especially of Japanese descent, usually between the ages of 1 and 8. In 20% of untreated patient, the coronary arteries can be affected by the vasculitis. The cardiac involvement can cause acute myocarditis with heart failure, arrhythmias, pericarditis, endocarditis, and sometimes coronary artery aneurysms. Acquired heart disease in children is predominantly cause by this disease. Other areas of the body affected by the inflammation are the kidneys, biliary tract, upper respiratory tract, and pancreas. Only 2% of patients will have a reoccurrence, which will usually occur months to years later. (Merck Manual)

     The prognosis is generally good for patient’s who are able to get treatment early enough, although there is the possibility of serious cardiovascular problems developing later on in life. For those who develop a large aneurysm (larger diameter than 8 mm) have the worst prognosis, as those will probably not regress or resolve. In some patients, severe ischemic heart disease can arise, which may necessitate a heart transplant. (pathology)

Prevalence

     Kawasaki Disease can be found in any ethnic group, but is predominately found in the Asian population, more specifically the Japanese. (pathology) Japanese children under the age of 5 are 10 times more likely than Caucasian children of the same age group. The incidence of this disease in Hispanic and African American children falls in the middle. Males are 1.5 times more likely to get Kawasaki Disease than females. In the United States, there are approximately 4,000 cases each year. (Boston Children’s Hospital) Eighty percent of the cases of Kawasaki Disease are found in children under the age of 5. (pathology) The most prevalent age group is 18-24 months, and it is rare under the age of 4 months, (Merck Manual)

Characteristics/Clinical Presentation

     Kawasaki Disease begins with a fever that lasts for at least 5 days. Along with the fever the child may display irritability, intermittent fatigue, and occasional colicky abdominal aching. (Merck Manual) During the acute stage, which occurs during the first 2 weeks, there is inflammation of the vascular system and the immune system has been activated. During this time, the arterioles, venules, and capillaries are affected. Later on in the disease the main coronary arteries, the heart, and the larger veins will be affected. Also during the acute phase, there are circulating antibodies that are cytotoxic to vascular endothelial cells, and the anticardiac myosin autoantibodies may be elevated. The elevation of the anticardiac myosin autoantibodies may be responsible for the myocardial damage that occurs. (pathology)One to 2 days after the start of the fever, bulbar conjunctiva will appear bilaterally, without exudate. About 5 days after the onset of the fever, a polymorphous, erythematous macular rash appears most commonly over the trunk, but is typically concentrated in the perineal area. At this point the mouth is characterized as having a reddened tongue, dry, cracked, and fissured lips, and infected pharynx. Between the 3rd and 5th day, the palms of the hands and soles of the feet will begin to show red-purple discoloration and be slightly edematous. Pallor of the proximal parts of the fingernails and/or toenails may appear within 7 days after the start of Kawasaki Disease. On the 10th day, desquamation of the palmar, plantar, perineal, and periungual regions will be present. Sometimes the skin will flake off in large pieces, and the skin underneath will appear normal. In half of the patients affected by Kawasaki Disease, swollen and tender cervical lymph nodes will be present. Other findings present with this disease are arthalgias, urethritis, aseptic meningitis, hepatitis, vomiting, diarrhea, otitis, and hydrops of the gall bladder, and anterior uveitis. Cardiac complications usually begin about 1-4 weeks after the onset of the initial symptoms. (Merck Manual)
     Clinical manifestation: (pathology, Merck Manual)
          • Acute Phase:
               • Abrupt high fever lasting more than 5 days that will not respond to antibiotics or antipyretics
               • Extreme irritability
               • Fatigue
               • Abdominal pain
          • Sub-acute Phase:
               • Fever resolves
               • Irritability continues
               • Anorexia
               • Rash of the trunk and extremities with a reddened palmar surface of the hands and feet
               • Desquamation of the tips of the fingers and toes
               • Peripheral edema of the hands and feet
               • Pallor of the fingernails and/or toenails
               • Cervical lymphadenopathy (typically unilateral)
               • Conjunctival infection without exudate (bilateral)
               • Changes in the oral mucous membranes, such as erythema, dryness or cracking of the lips, and reddening of tongue
               • The following are signs/symptoms found in 1/3rd of all cases:
                    o Arthalgias
                    o Gastrointestinal tract symptoms
                    o Cardiac involvement, such as myocarditis, pericarditis, and arteritis. These can predispose one with Kawasaki Disease to the development of coronary artery aneurysm. This occurs in 25% of the cases that are not treated within 10 days of the onset of the fever.
          • Convalescent Phase:
               • All of the clinical signs and symptoms have resolved
               • The blood values are still abnormal

Associated Co-morbidities

Currently there are no associated comorbidities with Kawasaki Disease, but there are possible risks that can develop after having the disease. Twenty percent of cases will go on to develop cardiac complications, while there is also a small risk of developing Reyes Disease (pathology, Merck Manual)

Medications

The medications use to treat Kawasaki Disease include:
     • High dose IV immune globulin (IVIG)
     • High-dose aspirin
     • Anticoagulants if complications develop
The IVIG is used to control the involvement of the coronary arteries, while the high-dose aspirin is used to lower the child’s fever, control the inflammation happening throughout the body, and to also decrease the chance of developing coronary artery abnormalities. For children with large coronary artery abnormalities other anticoagulant therapy will be needed, such as warfarin or dipyridamole (pathology, Merck Manual)

Diagnostic Tests/Lab Tests/Lab Values

     The diagnosis of Kawasaki Disease is made based off the clinical signs and symptoms of the patient, laboratory tests, a serial ECG and echocardiogram. There are no specific laboratory tests used to diagnose Kawasaki Disease, but they tend to check the child’s complete blood count (CBC), erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA), rheumatoid factor (RF), and throat and blood cultures. These laboratory tests are done mainly to exclude other diseases. The ECG and echocardiogram are done to allow the doctors to establish a baseline, and screen the patient’s myocardial and coronary artery status (pathology, Merck Manual)
Criteria for diagnosing Kawasaki Disease: (Merck Manual)
     • The diagnosis is made if the child has a fever 5 or more days and if 4 of the following 5 conditions are met:
     • Bilateral non-exudative conjuntival infection
     • Changes in the lips, tongue, or oral mucosa (infection, drying, fissuring, red strawberry tongue)
     • Changes in the peripheral extremities (edema, erythema, desquamation)
     • Polymorphous truncal exanthema
     • Cervical lymphadenopathy (at least 1 node > 1.5 cm in diameter)
Leukocytosis, an increase in immature white blood cells, is commonly found acutely. Also found in the laboratory tests can be mild normocytic anemia, thrombocytosis, elevated ESR or C-reactive protein. When other organ systems are involved, they may find sterile pyuria, elevated liver enzymes, cerebrospinal fluid pleocytosis, and proteinuria. (Merck Manual)

Etiology/Causes

     The cause of this disease is currently unknown, but due to seasonal and geographic outbreaks, there is thought to be an infectious etiology. The infectious cause is thought to be an immunologic reaction to an “infectious, toxic, or antigenic substance”. It is thought that a genetically predisposed child comes in contact with an infection or has an abnormal immunologic response to an infection, and that combination causes Kawasaki Disease. (pathology)

Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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