M30.3 - Mucocutaneous lymph node syndrome [Kawasaki]

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Acute febrile mucocutaneous lymph node syndrome
• Aneurysm of coronary artery due to and following acute febrile mucocutaneous lymph node syndrome
• Aneurysm of coronary vessels
• Myocarditis due to Kawasaki disease
• Primary systemic arteritis
• Vasculitis of medium sized vessel

Clinical Information

• Mucocutaneous Lymph Node Syndrome-. an acute, febrile, mucocutaneous condition accompanied by swelling of cervical lymph nodes in infants and young children. the principal symptoms are fever, congestion of the ocular conjunctivae, reddening of the lips and oral cavity, protuberance of tongue papillae, and edema or erythema of the extremities.

Diagnostic Related Groups - MS-DRG Mapping

The is diagnosis code is grouped in the following groups for version MS-DRG V40.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC). applicable from 10/01/2022 through 09/30/2023.

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Patient Education

Kawasaki Disease

What is Kawasaki disease?

Kawasaki disease is a rare illness that usually affects small children. Other names for it are Kawasaki syndrome and mucocutaneous lymph node syndrome. It is a type of vasculitis, which is inflammation of the blood vessels. Kawasaki disease is serious, but most children can fully recover if they are treated right away.

What causes Kawasaki disease?

Kawasaki disease happens when the immune system injures the blood vessels by mistake. Researchers do not fully know why this happens. But when it does, the blood vessels become inflamed and can narrow or close off.

Genetics may play a role in Kawasaki disease. There may also be environmental factors, such as infections. It does not seem to be contagious. This means that it cannot be passed from one child to another.

Who is at risk for Kawasaki disease?

Kawasaki disease usually affects children under the age of 5. But older children and adults can sometimes get it. It is more common in boys than girls. It can affect children of any race, but those with Asian or Pacific Islander descent are more likely to get it.

What are the symptoms of Kawasaki disease?

The symptoms of Kawasaki disease may include:

• High fever lasting at least five days
• A rash, often on the back, chest, and groin
• Swollen hands and feet
• Redness of the lips, lining of the mouth, tongue, palms of the hand, and soles of the feet
• Pink eye
• Swollen lymph nodes

What other problems can Kawasaki disease cause?

Sometimes Kawasaki disease can affect the walls of the coronary arteries. These arteries bring supply blood and oxygen to your heart. This can lead to:

• An aneurysm (bulging and thinning of the walls of the arteries). This can raise the risk of blood clotss in the arteries. If the blood clots are not treated, they could lead to a heart attack or internal bleeding.
• Inflammation in the heart
• Heart valve problems

Kawasaki disease can also affect other parts of the body, including the brain and nervous system, the immune system, and the digestive system.

How is Kawasaki disease diagnosed?

There is no specific test for Kawasaki disease. To make a diagnosis, your child's health care provider may use many tools:

• A physical exam, which includes looking at the signs and symptoms
• Blood and urine tests to rule out other diseases and check for signs of inflammation
• Tests to check for damage to the heart, such as an echocardiogram and electrocardiogram (EKG)

What are the treatments for Kawasaki disease?

Kawasaki disease is usually treated in the hospital with an intravenous (IV) dose of immunoglobulin (IVIG). Aspirin may also be part of the treatment. But do not give your child aspirin unless the health care provider tells you to. Aspirin can cause Reye syndrome in children. This is a rare, serious illness that can affect the brain and liver.

Usually treatment works. But if it is not working well enough, the provider may also give your child other medicines to fight the inflammation. If the disease affects your child's heart, he or she might need additional medicines, surgery, or other medical procedures.

Kawasaki disease

Kawasaki disease is a sudden and time-limited (acute) illness that affects infants and young children. Affected children develop a prolonged fever lasting several days, a skin rash, and swollen lymph nodes in the neck (cervical lymphadenopathy). They also develop redness in the whites of the eyes (conjunctivitis) and redness (erythema) of the lips, lining of the mouth (oral mucosa), tongue, palms of the hands, and soles of the feet.

Without treatment, 15 to 25 percent of individuals with Kawasaki disease develop bulging and thinning of the walls of the arteries that supply blood to the heart muscle (coronary artery aneurysms) or other damage to the coronary arteries, which can be life-threatening.

Code History

• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)