Introduction: Acute phase Kawasaki disease (KD) is often associated with myocarditis. In most cases, such myocarditis is self-limiting. Fulminant myocarditis is rare as a cause of death for acute KD. We report a case of fatal KD associated with fulminant myocarditis.
Case: A 4-year-old boy presented high fever and consulted family doctors for the second successive day. Although he received antibiotics, high fever persisted. He consulted to our hospital on the 4th day of illness. At the initial visit, conjunctival congestion, redness of pharyngeal and lip, bilateral cervical lymphadenitis and erythema on neck, palms and abdomen were observed. Initial laboratory data were shown as follows: WBC count 20,800 / μL, CRP 14.18 mg / dL. He was hospitalized with the diagnosis of acute febrile disease including KD. At first he was prescribed acetylsalicyclic acid and intravenous infusion of antibiotics, however high fever still persisted. On the 6th day of illness, immunoglobulin (IG) and prednisolone were administered intravenously because he was anticipated to be refractory to IG. On echocardiography, left ventricular contraction was normal. There were no findings of coronary artery abnormalities or pericardial effusion. After the first administration of IVIG, high fever persisted. Inflammatory markers on blood examination exacerbated. Laboratory data on the 8th day of illness were as follows: WBC 23,500 / μL, CRP 27.35 mg / dL, CPK 1,451 IU / L (MB 2%). Therefore, we added the second IVIG, steroid pulse therapy and urinastatine. On the next day (9th day), however, he suddenly suffered from cardiopulmonary arrest and deceased.
Pathological findings revealed scattered myocardial necrosis on both ventricles. The CD 3, CD 8 positive T lymphocytes were infiltrated around myocytes. There were no findings of coronary artery aneurysms or thromboembolisms. These findings were compatible with fulminant myocarditis.
Discussion: Recent advances in therapy for acute KD significantly reduce coronary artery lesions and subsequent mortality. However, clinical feature of KD has a lot of variety. Although fulminant myocarditis associated with KD in early acute phase is extremely rare, we should prepare for refractory KD and its abrupt change.