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Things You Didn’t Know About Kawasaki Disease and Symptoms

Things You Didn’t Know About Kawasaki Disease and Symptoms

Kawasaki Disease and Symptoms

We’ll be telling you what you need to know about Kawasaki disease, the symptoms, and how to prevent yourself from the virus. At first, you need to ask yourself, what is Kawasaki disease? It is simply a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age. Clearly stating; it is a form of vasculitis, where blood vessels become inflamed throughout the body, where the fever typically lasts for more than five days and is not affected by usual medications. Kawasaki disease is rare. It affects between 8 and 67 per 100,000 people under the age of five except in Japan, where it affects 124 per 100,000. Boys are more commonly affected than girls. The disorder was first described in 1967 by Tomisaku Kawasaki in Japan. Adult-onset of Kawasaki disease is rare. The presentation differs between adults and children: in particular, it seems that adults more often have cervical lymphadenopathy, hepatitis, and arthralgia. Now read further below to still know about Kawasaki disease.

A question was raised about whether the disease only started during the period between 1960 and 1970, but later a preserved heart of a seven-year-old boy who died in 1870 was examined and showed three aneurysms of the coronary arteries with clots, as well as pathologic changes consistent with Kawasaki disease. One thing to know about Kawasaki disease is that it’s now recognized across worldwide. Why cases began to emerge across all continents around the 1960s and 1970s is unclear. Possible explanations could include confusion with other diseases such as scarlet fever, and easier recognition stemming from modern healthcare factors such as the widespread use of antibiotics.

Things You Didn’t Know About Kawasaki Disease and Symptoms
Things You Didn’t Know About Kawasaki Disease and Symptoms

To Further Still Know About Kawasaki Disease

Before we further highlight things you to know about Kawasaki disease, we’ll first of all have to narrate the history of the virus.

A Brief History about Kawasaki Disease

The disease was first reported by Tomisaku Kawasaki in a four-year-old child with a rash and fever at the Red Cross Hospital in Tokyo in January 1961, and he later published a report on 50 similar cases. Later, Kawasaki and colleagues were persuaded of definite cardiac involvement when they studied and reported 23 cases, of which 11 (48%) patients had abnormalities detected by an electrocardiogram. In 1974, the first description of this disorder was published in the English-language literature. In 1976, Melish et al. described the same illness in 16 children in Hawaii. Melish and Kawasaki had independently developed the same diagnostic criteria for the disorder, which are still used today to make the diagnosis of classic Kawasaki disease. Dr. Kawasaki died on June 5th, 2020 at the age of 95. In the United States and other developed nations, Kawasaki disease appears to have replaced acute rheumatic fever as the most common cause of acquired heart disease in children.

See Also: Things You Didn’t Know About Coronavirus

What Causes Kawasaki Disease?

While the cause is unknown, it may be due to an infection triggering an autoimmune response in those who are genetically predisposed. It does not spread between people. Diagnosis is usually based on a person’s signs and symptoms.

  • Other tests such as an ultrasound of the heart and blood tests may support the diagnosis.
  • Other conditions that may present similarly include scarlet fever and juvenile rheumatoid arthritis, as well as a multisystem inflammatory syndrome in children.
  • Typically, the initial treatment of Kawasaki disease consists of high doses of aspirin and immunoglobulin.
  • Usually, with treatment, fever resolves within 24 hours and full recovery occurs. If the coronary arteries are involved, ongoing treatment or surgery may occasionally be required.
  • Without treatment, coronary artery aneurysms occur in up to 25% and about 1% dies. 
  • With treatment, the risk of death is reduced to 0.17%.

What are the Common Symptoms of Kawasaki Disease?

Some of the symptoms that come as a result of this disease are as follows:

  • Large lymph nodes in the neck.
  • A rash in the genital area, and red eyes, lips, palms, or soles of the feet.
  • Within three weeks of the onset, the skin from the hands and feet may peel, after which recovery typically occurs.
  • In some children, coronary artery aneurysms form in the heart.
  • High fever (above 101 F) that lasts more than 5 days. It won’t go down even if a child takes medication that usually works on fever.
  • Irritated throat, mouth, and lips
  • Swollen, bright red “strawberry tongue”

In the second phase, symptoms include:

  • Joint pain
  • Belly pain
  • Stomach trouble, such as diarrhea and vomiting
  • Peeling skin on hands and feet

Other reported nonspecific symptoms include cough, rhinorrhea, sputum, headache, and seizure.

What is the Cause of this Disease?

  • The specific cause of Kawasaki disease is unknown. One possible explanation is that it may be due to an infection triggering an autoimmune response in children who are genetically predisposed. Other explanations exist. The pathogenesis is complex and incompletely understood.
  • Some circumstantial evidence points to an infectious cause. Despite an intense search, no specific pathogen has been identified. A debate has occurred as to whether a conventional antigenic substance or a superantigen may be implicated. The current consensus favors a response to a conventional antigen.
  • Data have revealed a correlation between Kawasaki disease and tropospheric wind patterns; winds blowing from Central Asia correlate with Kawasaki disease cases in Japan, Hawaii, and San Diego. This association with tropospheric winds has been shown to be modulated at seasonal and interannual timescales by the El Niño–Southern Oscillation phenomenon, suggesting a possible involvement of a wind-borne pathogen.
  • Efforts have been made to identify a possible pathogen in air-filters flown at an altitude above Japan. One study suggests one source being in northeastern China.

Diagnosis

Kawasaki disease can be diagnosed only clinically (i.e., by medical signs and symptoms). No specific laboratory test exists for this condition. Establishing the diagnosis is difficult, especially early in the course of the illness, and frequently children are not diagnosed until they have seen several health-care providers. Many other serious illnesses can cause similar symptoms and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury poisoning (infantile acrodynia).

Your doctor will do a physical exam and ask about your child’s symptoms. They’ll look for a long-lasting fever and at least four of these five signs:

  • Red eyes
  • Red lips and mouth
  • Red, swollen limbs
  • Rash
  • Swollen lymph nodes

They may need to do tests to rule out other illnesses or to see whether the condition has affected your child’s heart. These include:

  • Heart tests such as an electrocardiogram (EKG) and echocardiogram
  • Blood tests
  • Imaging tests like X-rays and coronary angiogram

In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.

How Can a Patient be Treated Then?

  • Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric cardiology, pediatric rheumatology, and pediatric infectious disease specialists (although no specific infectious agent has yet been identified).
  • To prevent damage to the coronary arteries, treatment should be started as soon as the diagnosis is made.
  • Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease and is administered in high doses with marked improvement usually noted within 24 hours. If the fever does not respond, an additional dose may have to be considered. In rare cases, a third dose may be given to the child. IVIG by itself is most useful within the first seven days of onset of fever, in terms of preventing coronary artery aneurysm. IVIG given within the first 10 days of the disease reduces the risk of damage to the coronary arteries in children, without serious adverse effects.
  • Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some) but salicylates alone are not as effective as IVIG. There is limited evidence to indicate whether children should continue to receive salicylate as part of their treatment. Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home, usually for two months to prevent blood clots from forming. Except for Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended for children due to its association with Reye syndrome. Because children with Kawasaki disease will be taking aspirin for up to several months, vaccination against varicella and influenza is required, as these infections are most likely to cause Reye syndrome. High-dose aspirin is associated with anemia and does not confer a benefit to disease outcomes.
  • About 15-20% of children following the initial IVIG infusion show persistent or recurrent fever and are classified as IVIG-resistant. While the use of TNF alpha-blockers (TNF-α) may reduce treatment resistance and the infusion reaction after treatment initiation, further research is needed.
  • Corticosteroids have also been used, especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome. Additionally, corticosteroid use in the setting of Kawasaki disease is associated with an increased risk of coronary artery aneurysm, so its use is generally contraindicated in this setting.
  • In cases of Kawasaki disease refractory to IVIG, cyclophosphamide and plasma exchange have been investigated as possible treatments, with variable outcomes. However, a Cochrane review published in 2017 found that, in children, the use of corticosteroids in the acute phase of KD was associated with improved coronary artery abnormalities, shorter hospital stays, a decreased duration of clinical symptoms, and reduced inflammatory marker levels. Patient populations based in Asia, people with higher risk scores, and those receiving longer steroid treatment may have greater benefit from steroid use.

With early treatment, rapid recovery from the acute symptoms can be expected, and the risk of coronary artery aneurysms is greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater, even many years later.