Pulse methylprednisolone with gammaglobulin as an initial treatment for acute Kawasaki disease

Abstract

Approximately 15-20% of patients with Kawasaki disease (KD) are not responsive to high-dose intravenous gammaglobulin (IVIG). We have previously reported a predictive method for identifying IVIG-non-responsive patients (high-risk KD patients). We determined the safety and effectiveness of pulse methylprednisolone with high-dose IVIG (mPSL+IVIG) as a primary treatment for high-risk KD patients. Sixty-two high-risk KD patients were treated with pulse methylprednisolone 30 mg/kg over 2 h, followed by IVIG 2 g/kg over 24 h (mPSL+IVIG group) and were compared with a historical control group of 32 high-risk patients treated with IVIG 2 g/kg alone at the participating hospitals before this study was opened (IVIG group). High-risk patients were identified with at least two of three predictors (C-reactive protein >or=7 mg/dL, total bilirubin >or=0.9 mg/dL or aspartate aminotransferase >or=200 IU/L). Sixty-six percent (95% confidence interval [CI] 54-78%) of patients had a prompt defervescence in the mPSL+IVIG group compared with 44% (95% CI 26-62%) for the IVIG group (p=0.048). Coronary artery lesions were observed in 24.2% (95% CI 13.2-35.2%) and 46.9% (95% CI 28.6-65.2%) of patients in the mPSL+IVIG and IVIG groups, respectively (p=0.025). This is the first report showing that mPSL+IVIG is effective and safe as a primary treatment for high-risk KD patients.

IL-1B Polymorphism in Association With Initial Intravenous Immunoglobulin Treatment Failure in Taiwanese Children With Kawasaki Disease

“…28 Okada et al also showed that methylprednisolone + IVIG is effective and safe as a primary treatment for high-risk KD patients. 29 However, Newburger et al's data do not provide support for the addition of a single, pulsed dose of intravenous methylprednisolone to conventional IVIG therapy as routine primary treatment of children with KD. 30 The usefulness of steroids in the treatment of KD is not well established.…”

Clinical Score and Transcript Abundance Patterns Identify Kawasaki Disease Patients Who May Benefit From Addition of Methylprednisolone

“…In these patients, CALs frequently developed, despite the use of additional treatments, including further IVIG and steroids, thus indicating that current alternative treatments are ineffective after the failure of the initial IVIG treatment (4 -6). In other words, an earlier and more effective primary therapy is required for IVIG-resistant patients (7)(8)(9)(10)(11). Steroid therapy has been reconsidered as a therapeutic strategy to treat KD (11)(12)(13)(14)(15).…”

“…In other words, an earlier and more effective primary therapy is required for IVIG-resistant patients (7)(8)(9)(10)(11). Steroid therapy has been reconsidered as a therapeutic strategy to treat KD (11)(12)(13)(14)(15). A novel therapeutic approach may not be necessary in all cases of KD because the appropriate dose of IVIG and aspirin yields a good response in 80 to 85% of patients.…”

Kawasaki Disease, Others Heart Injuries, Not Only Coronary Arteritis

“…The most utilized treatment is the application of Intravenous gammaglobulin (GGIV) at a dose of 2 per kg in a sole dose for a 12-infusion; this is the most accepted treatment and has allowed to the reduction of prevalence of aneurysms to <5% and a mortality of 2% to 0.3%, although the GGIV scheme is at a dosage of 400 mg × kg × day for 5 days, plus aspirin at 80 to 100 mg × kg × day. According to response, the GGIV dose can be repeated, or corticoids can be added, especially in refractory cases (Cha, 2008;Chung, 2009;Falcini, 2006;Hung, 2009;Miura, 2008;Newbauer, 2004;Ogata, 2009;Okada, 2009;Sano, 2010;Tremoulet, 2008).…”

“…Concerning recommendation for treatment of KD during the past 3 years, it as been mentioned that the greatest effectiveness in cases of relapse or resistance is obtained on utilizing pulses of methylprednisolone in addition to GG. In cases of recurrence of fever or resistance to GGIV, this combination has even been recommended as initial therapy (Cha, 2008;Chung, 2009;Miura, 2008;Ogata, 2009;Okada, 2009;Sha, 2008). Cardiovascular sequelae of KD in the adult in Japan.…”