A Report of Two Cases of Kawasaki Disease Treated With Plasma Exchange

Abstract

Kawasaki disease is a generalized vasculitis of unknown etiology that occurs predominantly in infants and young children. It is very important to prevent its cardiovascular manifestations, especially coronary artery lesions. Early treatment with intravenous immunoglobulin reduces cardiovascular sequelae, but some patients do not respond to this treatment, and they have a high incidence of coronary artery lesions. On the other hand, acute heart failure is rare in Kawasaki disease. We report on the cases of two patients with persistent fever and shock even after intravenous immunoglobulin therapy. In both cases, plasma exchange may have reduced the risk of coronary artery lesions and proved effective against acute heart failure with catecholamine-refractory shock; yet the mechanism of this improvement remains unclear.

Unusual manifestations of Kawasaki disease with retropharyngeal edema and shock syndrome in a Taiwanese child

“…22,23 Cardiogenic shock in KD could be caused by arrhythmia, ischemic heart disease, valvular disease, endocarditis, and myocarditis, but these complications were ruled out by echocardiography and electrocardiography. 12 The echocardiography of our patient revealed normal ejection fraction of the left ventricle (0.655), LCA dilatation and mitral regurgitation. Therefore, the possibility of cardiogenic shock in our patient was low.…”

“…3 Refractory KD may require repeat immunoglobulin, immunosuppressant, steroid, or plasma exchange treatment. 12,13 Patients with KD and shock seem to have higher incidences of IVIG resistance, and require additional anti-inflammatory treatment. 13 Our patient was fortunate and had a good response to the first dose of IVIG.…”

“…14 Inflammatory cytokines are known to damage myocardial cells and work as cardiac depressants. 12 In animal models, tumor necrosis factor-a and interleukin-6 have been reported to cause myocardial depression. 22,23 Cardiogenic shock in KD could be caused by arrhythmia, ischemic heart disease, valvular disease, endocarditis, and myocarditis, but these complications were ruled out by echocardiography and electrocardiography.…”

“…8 Cardiovascular manifestations include pericarditis, myocarditis, aortic root dilatation, valvulitis, coronary artery lesions such as dilatation, aneurysm and/or stenosis. 3,12 Some severely affected patients even die suddenly. The major complication of KD is coronary artery aneurysms, which develop in 25% of untreated patients making KD the leading cause of acquired heart disease among children in the developed world.…”

Advances in the treatment of Kawasaki disease

“…Two case series and a case report in Korea have documented success in using methotrexate (10 mg/body surface area weekly) in a total of 22 patients with IVIG-resistant disease [59][60][61]. Several case reports and one large retrospective case series (125 patients) in Japan have reported favorable outcomes for plasma exchange in IVIG-resistant disease [62][63][64][65][66]. Ulinastatin, a urinary trypsin inhibitor that protects tissues against neutrophil-mediated injury, has also regained some attention in the literature.…”