Acute Vasculitis Disease, also known as Kawasaki Disease, was first described in 1967 by a Japanese author named Kawasaki. This condition primarily affects children aged 2 to 5 years and has been reported in outbreaks in Japan, the United States, and Canada. The disease tends to have a strong onset during the winter and spring seasons.
Diagnosis is based on clinical findings that present as an acute condition resembling a childhood infection. A significant complication to be aware of is cardiac involvement, which carries the risk of coronary artery aneurysm in some cases.
In terms of pathophysiology, there is evidence of immune system and endothelial activation triggered by a superantigen from bacteria. However, the exact cause of this acute vasculitis remains unclear.
Kawasaki Syndrome, also referred to as the “acute lymphadenitis – skin – mucosal syndrome with fever”, includes the following symptoms:
– Fever is the initial symptom. The body temperature fluctuates between 38.5°C and 40°C, lasting on average 10 days, and neither antibiotics nor antipyretics are effective.
– Bilateral conjunctival inflammation appears during the first week.
– Mucosal lesions include red, dry, and cracked lips. The tongue is red, with diffuse erythema affecting the oral cavity and throat.
– Lesions on the extremities manifest as erythema and swelling of both hands and feet. The skin on the fingers may peel. There are various rashes on the trunk resembling non-itchy urticaria. Rash and skin peeling in the buttocks and perineal regions are suggestive diagnostic signs.
– Cervical lymphadenopathy: A large lymph node may present on one side of the neck. The node is tense, firm, and may regress within a few days but does not produce pus.
These are the typical symptoms. However, atypical forms can occur, often with severe manifestations, delayed diagnosis, and are seen in infants with a high risk of coronary artery aneurysm.
Regarding laboratory tests, they do not significantly assist in diagnosis since no specific tests are available. Bacterial and viral cultures typically yield negative results. Inflammatory syndrome increases the erythrocyte sedimentation rate and polymorphonuclear leukocyte count. Thrombocytosis usually occurs later. Moderate liver dysfunction may be present.
Kawasaki Disease progresses through three stages:
– Acute stage lasting 1-2 weeks.
– Subacute stage: Fever, rash, and cervical lymphadenopathy resolve while skin peeling around the nails occurs. During this stage, there is a risk of sudden death due to coronary artery complications.
– Complete recovery stage occurs approximately 70 days after the onset of the disease.
In terms of cardiac complications, the incidence is one in two children. Particularly severe is the occurrence of coronary artery aneurysms between days 10 to 25. Cardiac prognosis primarily depends on the size of the aneurysm. Large aneurysms are a potential cause of heart failure and even sudden death due to myocardial infarction. Moderate and small aneurysms may persist for years or regress (50% may disappear after 2 years).
Treatment
There is no specific treatment for Kawasaki Disease. Only gamma globulin has been shown to effectively alleviate clinical symptoms rapidly and prevent coronary artery complications. According to standard protocol, gamma globulin is administered at a dosage of 400 mg/kg/day for 4 days or a single dose of 2 g/kg continuously over 10 hours.
Aspirin, with its anti-inflammatory and antithrombotic properties, is the second mandatory treatment step, administered at a dosage of 100 mg/kg/day divided into 4 doses during the acute phase. In the recovery phase, low-dose aspirin is continued to prevent platelet aggregation if there are coronary artery aneurysms or persistent inflammatory syndrome.
Prognosis is closely related to early treatment based on a diagnosis established before day 7 to initiate high-dose gamma globulin therapy.
Kawasaki Disease is a leading cause of coronary artery disease in older children due to sequelae. Children need to be monitored at a center specializing in cardiovascular diseases.
