Management and outcome of intravenous gammaglobulin-resistant Kawasaki disease

Sittiwangkul R, Pongprot Y, Silvilairat S, Phornphutkul C
Correspondence: Dr Rekwan Sittiwangkul, rsittiwa@mail.med.cmu.ac.th

ABSTRACT
Introduction This study aimed to determine the prevalence and risk of intravenous gammaglobulin (IVIG)-resistant Kawasaki disease (KD) and report the outcome of treatment in patients with persistent or recurrent fever.
Methods 70 KD patients, who received IVIG treatment (2 g/kg) at a tertiary care hospital from January 1995 to June 2004, were retrospectively reviewed.
Results Nine (13 percent) of the 70 patients failed to respond to initial treatment with IVIG. The patients who did not respond to IVIG had higher erythrocyte sedimentation rate (ESR) (104 versus 74 mm/h; p-value is 0.003), longer total days of fever (14.4 +/- 3.8 versus 9.2 +/- 2.3 days; p-value is 0.003) and higher initial coronary artery lesions (CAL) (7 of 9 [77.7 percent] versus 10 of 61 [16.3 percent]; p-value is 0.001) than those who responded to initial treatment. Seven of the nine patients who were retreated with IVIG (2 g/kg) responded to the second dose. The remaining two patients (two of nine, 22 percent) had persistent fever, which subsided after two to three doses of pulse intravenous methylprednisolone. At two months follow-up, IVIG-resistant patients had higher CAL by echocardiogram than IVIG-responsive patients (33 percent versus 3.2 percent, p-value is less than 0.05). Two IVIG-resistant KD patients had delayed diagnosis and developed giant aneurysms.
Conclusion Patients with high ESR had increased risk of IVIG-resistant KD. IVIG-resistant Kawasaki patients had a higher prevalence of CAL at the acute phase and two months after onset.

Keywords: coronary artery lesion, gammaglobulin refractory Kawasaki disease, gammaglobulin resistance, intravenous immunoglobulin therapy, Kawasaki disease, methylprednisolone pulse therapy
Singapore Med J 2006; 47(9): 780-784