Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids

Wright, Dowain A.; Newburger, Jane W.; Baker, Annette L.; Sundel, Robert P.

doi:10.1016/s0022-3476(96)70447-x

Cited by 206 publications

(136 citation statements)

References 10 publications

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“…The overall resistance to the first dose of IVIG was 5.56%, similar to those of other studies, in which 5% to 30% of patients experienced a persistent fever despite initial IVIG and aspirin treatment. 3,[10][11][12][13][14] All patients who failed initial IVIG therapy received additional IVIG 2 g/kg therapy without aspirin, and 66.67% of these had defervescence within 3 days after the additional IVIG, similar to results of other studies with approximately two thirds of initial nonresponders responding to a second dose of IVIG plus aspirin. 12,15,16 The result of our study indicates that the use of aspirin in acute KD has no significant effect on preventing the failure of IVIG therapy.…”

Section: Discussionsupporting

confidence: 77%

Treatment of Acute Kawasaki Disease: Aspirin’s Role in the Febrile Stage Revisited

et al. 2004

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ABSTRACT. Objective. To evaluate the effect of treatment without aspirin in the acute phase of Kawasaki disease (KD) and to determine whether it is necessary to expose children to high-or medium-dose aspirin.Methods. A total of 162 patients who fulfilled the established criteria of acute KD between 1993 and 2003 were included in this retrospective study. All patients were treated with high-dose intravenous immunoglobulin (IVIG; 2 g/kg) as a single infusion without concomitant aspirin treatment. Low-dose aspirin (3-5 mg/kg per day) was subsequently prescribed when fever subsided. Patients who had defervescence within 3 days after the completion of IVIG treatment were classified as the IVIG-responsive group, and those whose fever persisted for >3 days were classified as the IVIG-nonresponsive group. The 162 patients were divided further into 2 groups: those who were treated with IVIG before illness day 5, and those who were treated after illness day 5. We compared the response rate of IVIG therapy, duration of fever, and incidence of coronary artery abnormalities (CAAs) between these groups.Results. A total of 153 patients were classified into the IVIG-responsive group, and 128 (83.66%) of them had defervescence within 24 hours after completion of IVIG therapy. Nine (5.56%) patients were classified into the IVIG nonresponsive group, and all received additional IVIG (2 g/kg) without aspirin. Six (66.67%) had defervescence within 3 days after additional therapy. Patients in the IVIG-nonresponsive group had a significantly higher incidence of CAAs than those in the IVIG-responsive group (25% vs 2.92%). In the group that was treated before illness day 5 (n ‫؍‬ 16), all patients had defervescence within 3 days after IVIG therapy and 13 (81.25%) had defervescence within 24 hours. In the group that was treated after illness day 5 (n ‫؍‬ 146), 137 (93.84%) patients had defervescence within 3 days and 115 (78.77%) had defervescence within 24 hours. One (6.67%) patient in the group that was treated before illness day 5 got a new onset of CAAs, as did 5 (3.85%) in the group that was treated after illness day 5. There was no statistically significant difference in the response rate of IVIG therapy, duration of fever, and incidence of CAAs between these 2 groups. Conclusion.The results of our study indicate that the treatment without aspirin in acute stage of KD had no effect on the response rate of IVIG therapy, duration of fever, or incidence of CAAs when children were treated with high-dose (2 g/kg) IVIG as a single infusion, despite treatment before or after day 5 of illness. We conclude that it seems unnecessary to expose children to high-or medium-dose aspirin therapy in acute KD when the available data show no appreciable benefit in preventing the failure of IVIG therapy, formation of CAAs, or shortening the duration of fever. K awasaki disease (KD) is an acute systemic vasculitis of unknown origin that occurs predominantly in children who are Ͻ5 years old. The most significant complication is coronary arteritis, and an...

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Section: Discussionsupporting

confidence: 77%

Treatment of Acute Kawasaki Disease: Aspirin’s Role in the Febrile Stage Revisited

et al. 2004

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“…In the last 6 years, the use of corticosteroids in KD reemerged with reports of 8 patients who had prompt, complete responses to IV pulse methylprednisolone. [2][3][4][5] Unlike these reported patients, 2 of our patients were unable to tolerate discontinuation of methylprednisolone without the symptoms of active KD reoccurring. The addition of cyclophosphamide in these 2 patients seemed to allow methylprednisone to be tapered and slowly discontinued.…”

Section: Discussionmentioning

confidence: 97%

“…Treatment failure was defined as return of fever and 1 or more of the initial symptoms that led to the diagnosis of KD within 2 to 7 days of treatment with IVIG. 2,3 No patient was referred to our center because of failed treatment elsewhere; we did not include patients with atypical KD.…”

Section: Methodsmentioning

confidence: 99%

“…Recently, corticosteroids have been efficacious in a small number of children who do not respond to initial IVIG treatment. [2][3][4][5] We were impressed with several severe cases of KD that were unresponsive to repeated doses of IVIG and that required a large amount of corticosteroid to suppress the inflammatory response. Therefore, we were interested in our institution's experience with KD patients unresponsive to IVIG.…”

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confidence: 99%

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Initial Intravenous Gammaglobulin Treatment Failure in Kawasaki Disease

et al. 2000

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ABSTRACT. Objectives. To determine initial intravenous gammaglobulin (IVIG) treatment failures in Kawasaki disease (KD) and to report the outcome of retreatment and our use of pulse intravenous (IV) methylprednisolone and cyclophosphamide in patients with persistent KD.Study Design. Retrospective analysis of the treatment and response of children with KD over 3 years.Results. Fifty (77%) of 65 patients completely responded to a single treatment with IVIG (2 g/kg). Fifteen patients (23%) required retreatment; 10 patients fully responded but 5 had persistent disease (3 developed coronary aneurysms and 4 developed coronary artery thrombosis). Four of these 5 patients with persistent disease were treated with pulse IV methylprednisolone and 2 were also treated with IV cyclophosphamide. There was no progression of coronary aneurysms and no deaths. No initial patient characteristics predicted IVIG treatment failure or the development of coronary aneurysms.Conclusion. Nearly 23% of patients with KD may require retreatment and 8% may develop coronary aneurysm. Additional antiinflammatory therapy, such as IV methylprednisolone and IV cyclophosphamide, may be helpful in treating persistent KD. Pediatrics 2000;105(6). URL: http://www.pediatrics.org/cgi/content/full/105/6/ e78; corticosteroids, vasculitis, Kawasaki disease, heart disease, gammaglobulin therapy, cyclophosphamide.

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“…In addition, cases of KD resistant to IVIG therapy have been reported (11,12). In Japan and the USA, 20-30% of KD cases have been reported to be IVIG-resistant (13). Therefore, a better understanding of the molecular mechanisms underlying KD is required in order to develop targeted treatments.…”

Section: Introductionmentioning

confidence: 99%

Screening of differentially expressed genes associated with Kawasaki disease by microarray analysis

Jiang

Cai

et al. 2017

Experimental and Therapeutic Medicine

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Abstract. Kawasaki disease (KD) is an autoimmune disorder that can induce coronary artery aneurysms, particularly in the case of delayed diagnosis and/or treatment. Early diagnosis is important for treatment and reduces the risk of heart injury. The aim of the present study was to identify differentially expressed genes by comparing the levels of gene expression in human umbilical vein endothelial cells following treatment with plasma from healthy individuals and patients with acute or convalescent KD. Following comparison of the control and acute KD groups, 385 up-regulated and 537 down-regulated genes were identified in the acute KD group. In the convalescent group, 505 and 879 genes were up-regulated and down-regulated, respectively, relative to the control group. Genes involved in the immune system and cell growth factors were up-regulated, while genes functioning in methylation were down-regulated, following treatment with KD plasma. In addition, five potential candidate molecular markers of KD, C-X-C motif chemokine ligand 2 (CXCL2), interleukin (IL) 8, tripartite motif containing 58 (TRIM58), immunoglobulin superfamily member 3 (IGSF3) and runt related transcription factor 1 (RUNX1) were identified by microarray analysis and verified using quantitative polymerase chain reaction. A significant positive correlation was identified between the neutrophil polys and expression levels of four of these candidate genes, including CXCL2, IL8, TRIM58, and IGSF3 (all P<0.01; R 2 ≥0.64). However, only CXCL2 expression was significantly positively correlated with neutrophil polys (P=0.01; R 2 =0.64) and neutrophil bands (P<0.001; R 2 =0.73).These results indicate that CXCL2 serves a crucial role in the injury of endothelial cells by KD plasma.

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Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids

Cited by 206 publications

References 10 publications

Treatment of Acute Kawasaki Disease: Aspirin’s Role in the Febrile Stage Revisited

Treatment of Acute Kawasaki Disease: Aspirin’s Role in the Febrile Stage Revisited

Initial Intravenous Gammaglobulin Treatment Failure in Kawasaki Disease

Screening of differentially expressed genes associated with Kawasaki disease by microarray analysis

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