Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease

Abstract

Background— Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ∼15% to 25% of untreated children and may lead to ischemic heart disease or sudden death. Methods and Results— A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for ≥5 days and ≤4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-α antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata. Conclusions— Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.

Risk factors of immunoglobulin resistance and coronary complications in children with Kawasaki disease

“…The incidence in Europe and among the Caucasians is significantly lower as compared to the epidemiological data from Japan, where it is estimated to be 8/100,000 in Europe in children < five years of age and 265/100,000 in Japan [1,3]. Our material confirms higher incidence in children < 60 months of age (85% of patients, median age 26 months), which is consistent with American observations (24-26 months) [2]. Our study also confirms a preponderance of male over female patients (2.3:1).…”

“…Neither American nor European guidelines have recommended corticosteroids as the first-line treatment [1,2]. Only one of our patients was given second IVIG therapy in addition to corticosteroids due to the recurrence of fever and persistently elevated markers of inflammation after the first infusion.…”

“…Currently the disease is considered one of the major causes of acquired heart disease in children in developed countries and it is the second commonest systemic vasculitis after Henoch-Schönlein purpura [1,2]. The highest incidence is reported in Japan and other countries of Far East Asia and also in individuals of Asian origin who live in different regions of the world [3].…”

Kawasaki disease: An evaluation of children in Erzurum

“…Staphylococcus aureus, Streptococcus pyogenes, Epstein-Barr virüs, parvovirüs B19, adenovirüs ve coronavirüs gibi patojenlerin gösterdiği bölgesel ve mevsimsel değişikliklerin bu dağılımda rolü olduğu düşünülmüştür (21) . Günümüzde KH tanısında ve yönetiminde, 2004 yılında güncellenmiş olan Amerikan Kalp Cemiyetinin (AHA) ve 2010'da yayınlanan Japon Dolaşım Sistemi Cemiyeti Çalışma Grubunun rehberleri kullanılmak-tadır (10,22) . Tanı kriterleri içerisinde ateş ana kriterdir.…”

“…Hastalar bu kriterleri sağlıyorsa, komplet KH; en az 5 gün süren ateşe 4'ten az sayıda kriter eşlik ediyorsa inkomplet KH olarak tanımlandı. Semptomlar başladıktan sonra hastaneye başvuru süresine göre ilk on günde başvuran olgular akut, onuncu günden sonraki başvurular subakut olarak sınıflandırıldı (10) . Tüm hastalar için, koroner arter tutulumunu tahmin etmede kullanılan ve 7 kriterden (beyaz küre sayısı >12,000/mm 3 , trombosit sayısı <350,000/mm 3 , CRP>3 mg/dL, hematokrit <%35, albümin <3,5 g/ dL, yaş<12 ay ve erkek cinsiyet) oluşan skorun 4 ve üzerinde olması durumunda, hastada koroner arter anevrizması gelişme riskinin yüksek olduğu düşünü-len Harada skorlaması yapıldı (11) .…”