Infliximab Plus Plasma Exchange Rescue Therapy in Kawasaki Disease

Sonoda, Kaori; Mori, Masaaki; Hokosaki, Tatsunori; Yokota, Shumpei

doi:10.1016/j.jpeds.2014.01.020

Cited by 52 publications

(29 citation statements)

References 17 publications

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“…In the Japanese trial, 20 KD patients resistant to 2 consecutive IVIG infusions (2 g/kg each) were treated with infliximab (5 mg/ kg), and an apparent clinical response was achieved in 18 (90%). 229 The 2 unresponsive patients were treated with plasma exchange with resolution of their inflammation. The coronary artery abnormalities detected by echocardiogram all subsequently resolved.…”

Section: Infliximabmentioning

confidence: 99%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

McCrindle¹,

Rowley²,

Newburger³

et al. 2017

Circulation

2,870

4,124

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BACKGROUND:Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. METHODS AND RESULTS:To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and longterm outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. CONCLUSIONS:These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. K awasaki disease (KD) is an acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age. When initially described, the potential for coronary artery complications was not appreciated. KD is now the most common cause of acquired heart disease in children in developed countries. In the absence of pathognomonic tests, the diagnosis continues to rest on the identification of principal clinical findings and the exclusion of other clinically similar entities with known causes. Timely initiation of treatment with intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery aneurysms defined from absolute luminal dimensions from 25% to ≈4%. Ongoing studies with additional therapies have not substantially reduced this residual risk. The long-term prognosis is determined by the initial and current level of coronary artery involvement. Certain subsets of patients are at risk for myocardial ischemia from coronary artery thrombosis and stenoses. Medical management of such patients hinges on judicious use of thromboprophylaxis and vigilance to identify evolving stenoses. Invasive revascul...

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Section: Infliximabmentioning

confidence: 99%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

McCrindle¹,

Rowley²,

Newburger³

et al. 2017

Circulation

2,870

4,124

View full text Add to dashboard Cite

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“…Importantly, no infections were attributed to the use of anakinra [18]. Infliximab may also play a role in lowering inflammation in KD and potentially reducing coronary artery abnormalities [28,29]. …”

Section: Discussionmentioning

confidence: 99%

High dose anakinra for treatment of severe neonatal Kawasaki disease: a case report

2014

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We report an 11-week-old female who presented with Kawasaki disease (KD) complicated by macrophage activation syndrome (MAS). The infant presented to the hospital with persistent fever, cough, diarrhea, and emesis, among other symptoms. Her condition quickly began to decompensate, and she developed classic features (conjunctivitis, rash, cracked lips, distal extremity edema) prompting a diagnosis of acute KD. The patient was treated with standard therapy for KD including three doses of intravenous immunoglobulin (IVIG), aspirin, and high dose glucocorticoids with no change in her condition. Due to a high suspicion for MAS, high dose anakinra therapy was initiated resulting in dramatic clinical improvements. She also received one dose of infliximab for concern for coronary artery changes, and over the course of several months, anakinra and high dose glucocorticoids were tapered. Nearly complete reversal of echocardiogram changes were observed after 8 months, and the infant is now off all immunosuppressive therapy. In this case report, we briefly review the importance of early recognition of MAS in pediatric patient populations with rheumatic diseases, and we suggest early initiation of anakinra therapy as a rapid and effective treatment option.

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“…Two doses of IVIG followed by PE and IVMP from 10 th to 14 th day of illness resulted in considerable improvement in clinical symptoms as well as improvement of inflammatory markers, IL-6, sTNF αR type 1 and type 2 levels. Sonoda et al [83] used a stepwise treatment of IVIG, infliximab and PE to treat the KD patients. They selected 76 patients unresponsive to 2 doses of IVIG and treated them with infliximab first, followed by PE for those who did not even respond to infliximab (6 out of 76 patients).…”

Section: Treatmentmentioning

confidence: 99%

“…They selected 76 patients unresponsive to 2 doses of IVIG and treated them with infliximab first, followed by PE for those who did not even respond to infliximab (6 out of 76 patients). This resulted in the resolution of inflammation, with disappearance of fever and improvement of laboratory values [83]. Based on these findings, these investigators concluded that this stepwise protocol for refractory KD patients was highly effective.…”

Section: Treatmentmentioning

confidence: 99%

Kawasaki disease: etiopathogenesis and novel treatment strategies

Agarwal

Agrawal

2016

Expert Review of Clinical Immunology

164

113

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Introduction-Kawasaki disease is an acute febrile systemic vasculitis that predominantly occurs in children below five years of age. Its etiopathogenesis is still not clear, but it is thought to be a complex interplay of genetic factors, infections and immunity.Areas covered-This review article discusses in detail Kawasaki disease, with particular emphasis on the recent updates on its pathogenesis and upcoming alternate treatment options. Though self-limiting in many cases, it can lead to severe complications like coronary artery aneurysms and thrombo-embolic occlusions, and hence requires early diagnosis and urgent attention to avoid them. Intravenous immunoglobulin (IVIG) with or without aspirin has remained the sole treatment option for these cases, but 10-15% cases develop resistance to this treatment.Expert Commentary-There is a need to develop additional treatment strategies for children with Kawasaki disease. Targeting different steps of pathogenesis could provide us with alternate therapeutic options.

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Infliximab Plus Plasma Exchange Rescue Therapy in Kawasaki Disease

Cited by 52 publications

References 17 publications

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

High dose anakinra for treatment of severe neonatal Kawasaki disease: a case report

Kawasaki disease: etiopathogenesis and novel treatment strategies

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