KAWASAKI DISEASE [MUCOCUTANEOUS LYMPH NODE SYNDROME]

 KAWASAKI DISEASE 

[MUCOCUTANEOUS LYMPH NODE SYNDROME]

By, ASWINI, Pharm. D - MEDODRIX

• Kawasaki disease is 2^nd common vasculitis in children.

• It is a heart condition that develops suddenly. The condition causes the immune system to attack blood vessels, which become inflamed and swollen. It affects the coronary arteries [which carry blood to the heart muscle], lymph nodes, skin, and oral mucosa.

• It affects children less than 5 years and is more common in males.

CAUSE:

•    Exact cause is not known [idiopathic]
•    It may be caused due to infection or an inappropriate immune response to infection
•    Can be due to toxic substances released by bacteria [super antigens] that can trigger the immune response

PATHOPHYSIOLOGY:

CLINICAL FEATURES:

Fever persisting at least 5 days.

    Presence of at least 4 principal features:

1.  Acute: Erythema of palms, soles; edema of hands, feet.

     Subacute: Periungual peeling of fingers, toes.

2.  Polymorphous exanthema.

3.  Bilateral bulbar conjunctival injection.

4.  Lip cracking, strawberry tongue.

5.  Cervical lymphadenopathy.

DIAGNOSIS:

• Kawasaki disease is diagnosed based on a thorough clinical evaluation 
• a detailed patient history
• detection of characteristic features, including fever of at least five days and 
• at least four of five characteristic signs [reddened eyes; changes of the lips and mouth; reddish, swollen extremities; rash; and swollen lymph nodes]

LABORATORY FINDINGS:

• Leukocytosis with neutrophilia
• Elevated ESR
• Elevated CRP
• Anemia
• Abnormal plasma lipids

COMPLICATIONS:

• Coronary artery thrombosis
• Aneurysm

TREATMENT:

The goal of treatment is to lower fever and inflammation and prevent heart damage

Treatment for Kawasaki disease can include:

• Gamma globulin: Infusion of an immune protein (gamma globulin) through a vein (intravenously) can lower the risk of coronary artery problems. This helps to reduce inflammation in the vessels.    

• Aspirin: High doses treat inflammation. Aspirin can also decrease pain, joint inflammation, and fever. The aspirin dose will likely be lowered once the fever has been gone for 48 hours.

• Corticosteroids: Typically in patients unresponsive to standard therapies ( gamma globulin  and aspirin )

• Infliximab: In refractory cases with coronary aneurysms.

• Anticoagulants: In patients with large aneurysms in whom the risk of thrombus is high.