Pharmacological Therapy for Patients with Kawasaki Disease

Abstract

Kawasaki disease is a systemic vasculitis of unknown aetiology that has been reported worldwide since its initial description in Japanese children. The most significant sequelae of acute Kawasaki disease are related to the inflammation of small to medium sized arteries and, in particular, the development of coronary artery aneurysms. Because the aetiology is unknown, pharmacological therapy is nonspecific and directed towards modulation of the inflammatory response and inhibition of platelet activation with the aim of preventing coronary artery aneurysms. In the US, the recommended treatment for Kawasaki disease in the acute phase is a single, high dose of intravenous gammaglobulin (2 g/kg) and high dose aspirin (80 to 100 mg/kg/day). Use of this regimen has resulted in a significant decrease in the incidence of coronary artery abnormalities. Although the American Heart Association currently recommends high dose aspirin, moderate doses are used in Japan and the optimal dose of aspirin is not known. There has been renewed interest in the use of corticosteroids in the treatment of acute Kawasaki disease: however, their precise role remains unclear. Newer antiplatelet agents have also shown some promise in the treatment of patients with coronary artery aneurysms. Long term pharmacological therapy consists primarily of anticoagulation in patients with persistent coronary artery abnormalities. In this review, current recommendations for pharmacological therapy in Kawasaki disease are reviewed and some of the controversies in management of this disease, including management of patients who do not respond to initial therapy and the role of corticosteroids in the acute setting, are outlined.

Atypical Kawasaki disease

“…Since the etiology is unknown, pharmacological therapy is non-specific and directed towards modulation of the inflammatory response and inhibition of platelet activation with the aim of preventing coronary artery aneurysms [11]. Treatment should begin with intravenous immunoglobulin (IVIG) and high doses of aspirin as soon as the diagnosis is made.…”

“…The patient should also be given aspirin for its antiinflammatory and antiplatelet activity. In addition to aspirin, other antiplatelet drugs can be used such as clopidogrel and inhibitors of the platelet glycoprotein IIb/IIIa receptor [1,11]. For treatment of IVIG-resistant patients, a variety of therapies has been tried including repeated dose of IVIG, high-dose pulse methylprednisolone, cyclophosphamide or methotrexate, but there is no established guideline for the choice of treatment.…”

<b>Kawasaki Disease</b>

“…Nonspecific findings include anemia, neutrophilic leucocytosis, and thrombocytosis. 17,20) Thrombocytosis is typically more pronounced during the subacute phase of the illness. This thrombocytosis has been included as a minor characteristic feature of KD by Japanese workers.…”

The use of intravenous immunoglobulin in pediatric rheumatology

“…It can affect cor onary vessels leading to aneurysms and sudden cardiac death caused by ischemic heart disease. Efficacy of IVIG preparations in preventing developing coronary artery complications in children with KD is widely discussed and documented in the medical literature [15][16][17][18][19]. The efficiency of immunoglobulin infusions depends on the time since disease onset -initiating treatment within 10 days of the first symptoms is considered to be opti mal [3].…”