The immunomodulatory effects of intravenous immunoglobulin therapy in Kawasaki disease

Burns, Jane C.; Franco, Alessandra

doi:10.1586/1744666x.2015.1044980

Cited by 111 publications

(78 citation statements)

References 64 publications

(56 reference statements)

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“…Possible mechanisms of action include modulation of cytokine production, neutralization of toxins or other pathogenic agents, augmentation of regulatory T-cell activity, suppression of antibody synthesis, and provision of antiidiotypic antibodies. 161 Patients should be treated with IVIG 2 g/kg as a single infusion, usually given over 10 to 12 hours, together with ASA. 160 A variety of dose regimens have been used in Japan and the United States in the past.…”

Section: Intravenous Immunoglobulinmentioning

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

2,738

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: Intravenous Immunoglobulinmentioning

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

2,738

4,124

View full text Add to dashboard Cite

show abstract

“…The American Heart Association recommends a single administration of 2 g/kg of IVIG for KD patients within 5-10 days after onset of fever [39]. IVIG treatment not only reduces inflammation (fever, clinical signs, acute phase reactant levels) but also, and more importantly, prevents the development of coronary artery abnormalities [40][41][42][43][44]. It reduces the risk of coronary artery lesions from 20-25% to 2-4%.…”

Section: Therapeutic Intervention Standard Therapymentioning

confidence: 99%

Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease

Galeotti

Kaveri

Cimaz

et al. 2016

Drug Discovery Today

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Kawasaki disease (KD) is an acute febrile childhood inflammatory disease, associated with coronary artery abnormalities. The disease is believed to result from an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. KD is associated with an endothelial cell injury as a consequence of T cell activation and cytotoxic effects of various proinflammatory cytokines. Intravenous immunoglobulin (IVIG) infusion and aspirin are the standard treatment of acute KD. However, 10-20% of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis. The relative roles of second IVIG infusion, corticosteroids, calcineurin inhibitors, interleukin-1 antagonists and anti-tumor necrosis factor agents remain uncertain. In this review, we highlight the predisposing factors, pathogenesis and therapeutic intervention of KD, particularly new therapeutics for IVIG-resistant patients.

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“…Natural regulatory T cells (nTreg) are selected in the thymus during early development, recognize selfantigens, and play a critical role in maintaining immunological regulation. 12 Current data suggests that boosting T cell regulation is one of the most important functions of IVIG in the setting of KD. 13 Franco and colleagues recently demonstrated that a subset of nTreg that recognizes the heavy constant region of immunoglobulins (Fc), regulates vascular inflammation in KD.…”

Section: Intravenous Immunoglobulin (Ivig) Resistancementioning

confidence: 99%

Adjunctive therapies for Kawasaki disease

Campbell

Burns

2016

Journal of Infection

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The immunomodulatory effects of intravenous immunoglobulin therapy in Kawasaki disease

Cited by 111 publications

References 64 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

Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease

Adjunctive therapies for Kawasaki disease

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