Gammaglobulin Failure and Retreatment in Kawasaki Disease

Abstract

The objectives of this study were to evaluate the therapy protocol of gamma globulin (GG) re-treatment without steroids. Patients. From Jun. 1994 to Jun. 2001, 220 typical patients were enrolled within 8 days of illness. They had no coronary artery dilatation at admission and before GG treatment. Their risks were evaluated by the Iwasa's risk score system. Of 220 patients, 66% were selected as high-risk patients and 80% were treated with GG. Initial dose of GG was single 2g/kg for the high-risk patients and 1g/kg for the low-risk patients with prolonged fever, 49 patients had GG re-treatment. Maximum dose of GG was 8g/kg and mean dose of GG was 1.8g/kg per patient. Dose of re-treatment after Sep. 1996 was decided by WBC and CRP before and after GG treatment. Results. Of 220 patients, 13 patients had coronary artery lesions (CAL, over 3 mm in diameter) in acute stage and 3 patients had CAL at 30th days of illness, which were all transient dilatation. In high-risk female patients, 51 patients treated with GG had no CAL. In high-risk male patients from Jun. 1994 to Jun. 1999, 60 patients were treated with sulfonated GG (Sulfo-GG) or GG prepared by propylene glycol (PEG-GG). And CAL (8/31) in Sulfo-GG group was significantly higher than CAL (1/29) in PEG-GG group (pϭ0.017). In high-risk male patients after Jul. 1999, when we did not use Sulfo-GG, 1/22 had CAL. The other 3 CAL patients, whose CAL were under 3.1 mm in diameter, were high-risk patient without GG, low-risk patient without GG and high-risk patient with GG.

Response of refractory Kawasaki disease to intravenous methylprednisolone

“…2 For patients with immunoglobulin-resistant KD, retreatment with IVIG, methylprednisolone, cyclophosphamide and even infliximab have been found useful. [3][4][5][6] Most patients with refractory KD respond to a second dose of IVIG and rarely require further treatment. Two cases of KD are reported who developed CA aneurysms in spite of two courses of IVIG and subsequently responded to intravenous methylprednisolone.…”