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Kawasaki Disorder: Symptoms, Causes, Risk Factor & Diagnosis | Max Hospital

Kawasaki Disease: A Quick Guide to the Rare Disorder

By Dr. Munesh Tomar in Cardiac Sciences , Paediatric (Ped) Cardiology , Cardiology

Mar 15 , 2024 | 9 min read

Kawasaki Disease, although rare, is a potentially serious paediatric illness that demands prompt attention and intervention. Characterised by inflammation of blood vessels throughout the body, the disorder primarily affects young children, often presenting a unique set of symptoms that can be alarming to children as well as their parents. Despite its rarity, the significance of recognizing and promptly addressing the disorder cannot be overstated. In this guide, we delve into the essential aspects of Kawasaki Disease, including its symptoms, diagnosis, treatment, and the importance of early intervention in managing this health concern, starting with the basics. 

What is Kawasaki Disease?

Kawasaki Disease, also known as ‘mucocutaneous lymph node syndrome’, primarily affects children, especially those under the age of five. It causes inflammation in the walls of small and medium-sized arteries throughout the body, including the coronary arteries that supply blood to the heart. If left untreated, Kawasaki Disease can lead to complications, particularly affecting the coronary arteries, potentially resulting in weakening of the blood vessel walls. Early recognition and treatment, usually with intravenous immunoglobulin and aspirin, are crucial in managing the condition and reducing the risk of long-term heart complications. 

Causes and Risk Factors of Kawasaki Disease

Though the exact cause of Kawasaki Disease remains unknown, researchers believe that it might be triggered by an abnormal immune system response to an infection or another environmental factor in genetically predisposed individuals. Some factors are thought to play a role in the development of Kawasaki Disease. These include:

  • Genetic predisposition: There might be a genetic predisposition to Kawasaki Disease, as it tends to occur more frequently in certain ethnic groups, such as those of Asian descent, although it can affect children of all ethnicities.
  • Environmental triggers: The condition often follows viral or bacterial infections, although no specific infectious agent has been consistently identified as the cause. Some research suggests that the Disease might be triggered by an immune response to a viral or bacterial infection.
  • Age and sex: Kawasaki Disease primarily affects children, with a peak incidence in those under the age of five. Boys are slightly more likely than girls to develop Kawasaki Disease.
  • Seasonal variation: Cases of Kawasaki Disease often peak in the winter and early spring, suggesting a possible seasonal element to its occurrence.

Early Signs and Symptoms of Kawasaki Disease

Kawasaki Disease often develops in distinct phases, with symptoms evolving over time. Recognizing the early signs and symptoms of Kawasaki Disease is crucial for early diagnosis and prompt treatment. Some of the early signs and symptoms of Kawasaki Disease include:

  • Prolonged fever: One of the primary and earliest symptoms of Kawasaki Disease is a high and persistent fever that lasts for at least five days or more. The fever is typically unresponsive to usual fever-reducing medications.
  • Rashes: Children with Kawasaki Disease often develop a rash, which may appear as redness or a widespread, flat or raised rash that might involve the trunk, groyne area, and extremities. This rash may be more pronounced in the later stages of the illness.
  • Red eyes: Bilateral conjunctival injection, where the whites of the eyes become red or inflamed, is common. It's often described as "bloodshot" eyes without any discharge.
  • Changes in lips and mouth: The lips might become dry, cracked, or swollen. The mouth, throat, and tongue might also exhibit changes such as a strawberry-like appearance of the tongue, redness in the throat, or swollen, red, or cracked lips.
  • Swollen lymph nodes: Enlarged lymph nodes in the neck are common, appearing as lumps under the skin.
  • Changes in hands and feet: Children may experience swelling and redness of the hands and feet, often with peeling of the skin on the fingers and toes later in the illness.

Note: Not all children with Kawasaki Disease will experience every aforementioned symptom, and the combination and severity of symptoms can vary from person to person. If a child shows persistent fever and a constellation of these symptoms, especially when accompanied by irritability, joint pain, diarrhoea, or abdominal pain, it is crucial to seek immediate medical attention for further evaluation.

Complications of Kawasaki Disease

If left untreated or if not managed promptly, Kawasaki Disease can lead to various complications, particularly affecting the heart and blood vessels. These may include:

  • Coronary artery aneurysms: One of the most serious complications of Kawasaki Disease is the development of coronary artery aneurysms. The inflammation can weaken the walls of the coronary arteries, causing them to bulge or form aneurysms. These aneurysms can lead to blood clots, and in severe cases, they might even cause a heart attack.
  • Coronary artery vasculitis: Inflammation of the coronary arteries (vasculitis) can lead to changes in the blood vessel walls, potentially causing narrowing or obstruction. This can affect blood flow to the heart and increase the risk of heart complications.
  • Myocarditis and pericarditis: Kawasaki Disease can cause inflammation of the heart muscle (myocarditis) and the sac surrounding the heart (pericarditis), leading to complications such as abnormal heart rhythms, heart failure, or fluid accumulation around the heart.
  • Heart valve issues: In some cases, the inflammation from Kawasaki Disease can affect the heart valves, leading to regurgitation or stenosis, where the valves either leak or do not open or close properly.

Besides the aforementioned issues, Kawasaki Disease can also cause inflammation in the joints, liver, gallbladder, and in rare cases, the brain, leading to problems such as arthritis, hepatitis, gallbladder hydrops, or aseptic meningitis.

Prompt treatment of the disorder with intravenous immunoglobulin (IVIG) and aspirin within the first 10 days of illness significantly reduces the risk of complications, particularly those related to the heart. 

Diagnosis of Kawasaki Disease

The diagnosis of Kawasaki Disease can be challenging, as there are no specific tests to confirm the condition. Doctors typically rely on a combination of clinical criteria, medical history, physical examination, and certain laboratory tests to make a diagnosis. The key steps in diagnosing Kawasaki Disease include:

Evaluation of Symptoms

Physicians consider a constellation of symptoms that are characteristic of Kawasaki Disease. These include a prolonged fever of at least five days, along with other symptoms such as rash, red eyes, changes in the lips and mouth, swollen lymph nodes, and changes in the hands and feet.

Physical Examination

A thorough physical examination is conducted to assess the presence and progression of symptoms associated with Kawasaki Disease. The doctor pays close attention to any visible signs of the disease, including skin rashes, red eyes, swollen lymph nodes, and changes in the extremities.

Laboratory Tests

The doctor may also prescribe blood and urine tests to assess inflammation levels and look for other signs that support the diagnosis. These tests might include:

  • Complete Blood Count (CBC): To check for abnormalities in white blood cell count and haemoglobin levels.
  • C-Reactive Protein (CRP) and Erythrocyte Sedimentation Rate (ESR): These tests measure levels of inflammation in the body.
  • Liver Function Tests: To assess liver involvement.
  • Urinalysis: To check for signs of kidney involvement.

Echocardiogram

An ultrasound of the heart (echocardiogram) is often performed to check for potential damage to the coronary arteries and to monitor the heart's health. This test helps in assessing the risk of developing coronary artery aneurysms.

In addition to the aforementioned, regular follow-ups with a paediatric cardiologist are often recommended to monitor the heart's health. 

Treatment for Kawasaki Disease

The treatment for Kawasaki Disease aims to reduce inflammation, prevent complications, and alleviate symptoms. The treatment commonly includes:

Intravenous Immunoglobulin (IVIG): The cornerstone of treatment involves administering high doses of intravenous immunoglobulin, a solution containing antibodies to help reduce inflammation and modulate the immune response. This treatment is often given along with aspirin.

Aspirin therapy: High doses of aspirin are usually prescribed in the acute phase of the illness to reduce inflammation and prevent blood clotting. Aspirin is often given until the fever resolves, and then the dosage is reduced to prevent potential side effects.

Aspirin therapy: After the acute phase and fever have subsided, a low dose of aspirin might be recommended for a certain duration to reduce the risk of blood clots, particularly in cases where coronary artery aneurysms are present.

Monitoring and management of symptoms: Careful monitoring of the patient's condition, particularly the heart, is essential. Follow-up visits, tests, and sometimes echocardiograms are recommended to assess heart health and detect any potential complications.

Treatment for resistant or recurrent cases: In cases where the initial treatment with IVIG and aspirin does not resolve the symptoms or if the disease recurs, additional or alternative therapies might be considered. This can include a second dose of IVIG, corticosteroids, or other immunosuppressive medications.

Supportive care: During the acute phase of the illness, supportive care is essential to manage symptoms. This includes rest, adequate hydration, and monitoring for potential complications.

Final Words

Kawasaki Disease necessitates swift recognition and expert management to mitigate potential long-term complications. For comprehensive care and specialised attention, seeking guidance from experienced medical professionals is paramount. At Max Hospitals, our team of paediatric specialists and cardiologists offers tailored, state-of-the-art care, ensuring a meticulous approach from diagnosis to treatment and ongoing monitoring. We understand the critical importance of early intervention and personalised care to safeguard your child's health. Don't hesitate to consult the Kawasaki disease experts at Max Hospitals for the best possible guidance and care in managing Kawasaki Disease. 

Frequently asked Questions about Kawasaki Disease

Q. Can Kawasaki Disease be prevented?

There is no known way to prevent Kawasaki Disease, but early recognition, timely treatment, and close monitoring can significantly reduce the risk of complications.

Q. What is the long-term outlook for children with Kawasaki Disease?

With prompt treatment, the majority of children recover completely without long-term heart problems. However, regular follow-ups are essential to monitor heart health.

Absolutely! Here are ten more frequently asked questions about Kawasaki Disease along with their succinct answers:

Q. Is Kawasaki Disease contagious?

No, Kawasaki Disease is not contagious. It does not spread from person to person.

Q. What is the typical duration of Kawasaki Disease?

The acute phase of Kawasaki Disease often lasts around 1-2 weeks. The total duration can vary, and recovery timelines differ for each child.

Q. Can adults get Kawasaki Disease?

Although rare, Kawasaki Disease can affect adults. It's often more challenging to diagnose in adults due to different presentations and a lower incidence.

Q. Can a child have Kawasaki Disease more than once?

Recurrence of Kawasaki Disease is uncommon but can occur. Most cases experience it only once, but a small percentage might have a second episode.

Q. What are the potential side effects of the treatment for Kawasaki Disease?

Possible side effects from IVIG or aspirin are rare but can include allergic reactions, bleeding problems, or liver issues. These are closely monitored during treatment.

Q. Can children with a history of Kawasaki Disease participate in sports?

Typically, children who've recovered from Kawasaki Disease without complications can participate in sports and physical activities. However, guidance from a doctor is advisable.

Q. What should parents watch for after their child has been treated for Kawasaki Disease?

Parents should remain vigilant for any signs of recurrence of symptoms or potential complications, especially related to the heart, and attend scheduled follow-up appointments.

Q. Are there any dietary restrictions for children with Kawasaki Disease?

No specific dietary restrictions are typically recommended for Kawasaki Disease. However, if the child is taking aspirin, the doctor might suggest avoiding certain foods that could interact with the medication, such as foods high in salicylates.

Q. Is there a vaccine to prevent Kawasaki Disease?

Currently, there's no vaccine specifically designed to prevent Kawasaki Disease. Research in this area is ongoing.

Q. Can Kawasaki Disease cause long-term damage to the heart?

If left untreated or not managed promptly, Kawasaki Disease can lead to potential long-term complications affecting the heart, such as coronary artery aneurysms or other heart issues.

Q. Does having Kawasaki Disease as a child affect future health?

In most cases, children who've had Kawasaki Disease and received prompt treatment without significant heart complications have a good prognosis and typically do not have ongoing health issues related to the Disease in the long term.