A Combination Therapy for Kawasaki Disease with Severe Complications: a Case Report

Abstract

Kawasaki disease (KD) is a form of acute multisystem vasculitis that presents with various complications, including coronary artery aneurysm. Heart failure and brain damage are rare, but life-threatening complications are associated with KD. Here, we describe a 4-year-old girl who developed intravenous immunoglobulin-resistant KD with both left ventricular failure and acute encephalopathy. On day 8 of the illness, the low left ventricular ejection fraction, mitral regurgitation, and low blood pressure, which required continuous administration of dobutamine, were observed during the treatments for KD, including intravenous immunoglobulin. She also appeared unconscious, where the electroencephalogram showed slow waves of activity in all regions of the brain. The cardiac performance improved after she received plasma exchange for three days. However, her unconsciousness with slow waves of activity on electroencephalogram and fever continued after the plasma exchange. Therefore, she was treated with methylprednisolone pulse, followed by prednisolone, as well as intravenous immunoglobulin. Finally, she recovered without any cardiac or neurological sequelae not only at the time she was discharged, but also throughout the follow-up period. The combination therapy using plasma exchange and methylprednisolone pulse may be a treatment option for severe KD with left ventricular failure and acute encephalopathy complications.

Keywords: Combination therapy; Intravenous immunoglobulin-resistant Kawasaki disease; Methylprednisolone pulse; Plasma exchange.

Figure 1

Image examinations on day 8 of illness A: Chest X-ray The cardio-thoracic ratio was 0.53 and congestion was observed in the hilum of both lungs. B and C: Echocardiograms The left ventricular ejection function was 39%, percent fractional shortening was 20.9%, and the left ventricular end-diastolic dimension was 33.9 mm (104% larger than in a normal case).

Figure 3

Electroencephalograms A: Slow waves of activity in all brain regions, especially in the occipital lobe, were observed on day 8 of illness B: Abnormal waves were recovered on day 18 of illness.

Figure 3

Clinical course Treatments with IVIG (2 g/kg), aspirin (30 mg/kg/day), ulinastatin (5000 units/kg/dose, four times a day), and furosemide (1 mg/kg/dose, three times a day) were started on day 7 of illness. A continuous administration of dobutamine (2 μg/kg/min) was required, because of her low blood pressure. PE therapy was started on day 8 of illness because of left ventricular failure and acute encephalopathy. Due to the recurrence of the fever and EEG abnormalities, MP and IVIG were administered from day 11 of illness. Due to the combination therapy of IVIG, PE, and MP the symptoms of the patient improved. BT: body temperature, CK: creatine kinase, CRP: C-reactive protein, EEG: electroencephalogram, IVIG: intravenous immunoglobulin, KD: Kawasaki disease, MP: methylpredonine pulse therapy, NT-proBNP: N-terminal pro-brain natriuretic peptide, PE: plasma exchange, WBC: white blood cell.