The Treatment of Kawasaki Syndrome with Intravenous Gamma Globulin

Newburger, Jane W.; Takahashi, Masato; Burns, Jane C.; Beiser, Alexa S.; Chung, Kevin Kien Hoa; Duffy, C. Elise; Glodé, Mary P.; Mason, Wilbert H.; Reddy, Venudhar; Sanders, Stephen P.; Shulman, Stanford T.; Wiggins, James W.; Hicks, Ralph A.; Fulton, David R.; Lewis, Alan B.; Leung, Donald Y.M.; Colton, Theodore; Rosen, Fred S.; Melish, Marian E.

doi:10.1056/nejm198608073150601

Cited by 1,288 publications

(646 citation statements)

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“…However, administration of IVIg reduces coronary artery abnormalities such as aneurisms from 20% to 25% to 3% to 5% within 10 days of illness onset. 43 Recently, Lin et al 44 highlighted the role of HLA-E in the pathogenesis of KD. They measured plasma concentrations of soluble HLA-E (sHLA-E) by enzyme-linked immunosorbent assay in 96 Taiwanese patients with KD and in 93 healthy controls.…”

Section: Discussionmentioning

confidence: 99%

Therapeutic preparations of IVIg contain naturally occurring anti–HLA-E antibodies that react with HLA-Ia (HLA-A/-B/-Cw) alleles

Ravindranath

Terasaki

Pham

et al. 2013

Blood

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Section: Discussionmentioning

confidence: 99%

Therapeutic preparations of IVIg contain naturally occurring anti–HLA-E antibodies that react with HLA-Ia (HLA-A/-B/-Cw) alleles

Ravindranath

Terasaki

Pham

et al. 2013

Blood

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“…myasthenia gravis, and Kawasaki disease (16)(17)(18)(19). In this rcport, we describe the beneficial effccts of IV gamma globulin therapy on the clinical and laboratory features of systemic JRA.…”

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confidence: 99%

Intravenous gamma globulin therapy in systemic juvenile rheumatoid arthritis

Silverman

Laxer

Greenwald

et al. 1990

Arthritis & Rheumatism

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Intravenous (IV) gamma globulin has been successfully used as replacement therapy for antibody‐deficient patients and, more recently, in the treatment of autoimmune diseases such as idiopathic thrombocytopenic purpura, myasthenia gravis, and Kawasaki disease. In view of the successful treatment of these diseases, we initiated a pilot study of the effect of IV gamma globulin in systemic juvenile rheumatoid arthritis (JRA). Eight patients with active systemic JRA that was unresponsive to first‐line agents, second‐line agents, and/or corticosteroids received this therapy monthly for 6 months. Outcome measures included changes in articular and extraarticular features, steroid dosage, and laboratory parameters. Following IV gamma globulin therapy, there was significant improvement in arthritis and/or morning stiffness in 5 of 8 patients, while extraarticular features significantly improved in 7 of 8 patients. At study entry, 6 of 8 patients were receiving prednisone; at study end, prednisone was discontinued in 3 patients and decreased by more than 50% in the other 3. Overall, there was an 80% reduction in the prednisone dosage. Initially, all patients had anemia, low levels of serum albumin, and an elevated erythrocyte sedimentation rate, while a thrombocytosis was seen in 7 of 8 patients. Serum IgG was initially elevated in 6 patients. IV gamma globulin therapy resulted in a significant increase in hemoglobin and albumin levels and a significant decrease in the mean serum IgG level, platelet count, and erythrocyte sedimentation rate. In only 1 patient did IV gamma globulin fail to significantly improve the clinical or laboratory features of the disease. We suggest that this therapy may be beneficial in the treatment of systemic JRA.

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“…IVIG has now been proven to be efficacious for Kawasaki disease, idiopathic thrombocytopenic purpura, and polymyositisdermatomyositis. 7,8,22 The benefits seen in Kawasaki disease are particularly relevant to ARF, because the incidence of coronary artery aneurysms is significantly reduced by IVIG administration. A large variety of immunological mechanisms may be involved in the pathogenesis of ARF, including cross-reactive antibodies, release of cytokines, activation of T and B cells, and immune complex formation.…”

Section: Discussionmentioning

confidence: 99%

“…Recent work has indicated that intravenous immunoglobulin (IVIG) may be of benefit in immune-mediated cardiac disorders. 7,8 This is seen particularly in Kawasaki disease, in which the use of high-dose IVIG markedly reduces the prevalence of coronary artery abnormalities. 9 We present the results of a randomized, placebo-controlled trial of IVIG as an acute intervention in patients with ARF to determine whether there was a reduction in the extent and severity of carditis, more rapid resolution of inflammatory activity, or decreased chronic morbidity.…”

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confidence: 99%

Intravenous Immunoglobulin in Acute Rheumatic Fever

et al. 2001

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Background-Acute rheumatic fever (ARF) remains the leading cause of acquired heart disease in children worldwide. No therapeutic agent has been shown to alter the clinical outcome of the acute illness. Immunological mechanisms appear to be involved in the pathogenesis of ARF. Intravenous immunoglobulin (IVIG), a proven immunomodulator, may benefit cardiac conditions of an autoimmune nature. We investigated whether IVIG modified the natural history of ARF by reducing the extent and severity of carditis. Methods and Results-This prospective, double-blind, randomized, placebo-controlled trial evaluated IVIG in patients with a first episode of rheumatic fever, stratifying patients by the presence and severity of carditis before randomization. Patients were randomly allocated to receive 1 g/kg IVIG on days 1 and 2 and 0.4 g/kg on days 14 and 28, or they received a placebo infusion. Clinical, laboratory, and echocardiographic evaluation was performed at 0, 2, 4, 6, 26, and 52 weeks. Fifty-nine patients were treated, of whom 39 had carditis (including 4 subclinical) and/or migratory polyarthritis (nϭ39). There was no difference between groups in the rate of normalization of the erythrocyte sedimentation rate or acute-phase proteins at the 6-week follow-up. On echocardiography, 59% in the IVIG group and 69% in the placebo group had carditis at baseline. There was no significant difference in the cardiac outcome, including the proportion of valves involved, or in the severity of valvar regurgitation at 1 year. At 1 year, 41% of the IVIG and 50% of the placebo group had carditis. Conclusions-IVIG

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The Treatment of Kawasaki Syndrome with Intravenous Gamma Globulin

Cited by 1,288 publications

References 11 publications

Therapeutic preparations of IVIg contain naturally occurring anti–HLA-E antibodies that react with HLA-Ia (HLA-A/-B/-Cw) alleles

Therapeutic preparations of IVIg contain naturally occurring anti–HLA-E antibodies that react with HLA-Ia (HLA-A/-B/-Cw) alleles

Intravenous gamma globulin therapy in systemic juvenile rheumatoid arthritis

Intravenous Immunoglobulin in Acute Rheumatic Fever

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