Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study

Orenstein, Jan M.; Shulman, Stanford T.; Fox, Linda M.; Baker, Susan C.; Takahashi, Masato; Bhatti, Tricia R.; Russo, Pierre; Mierau, Gary W.; Chadarévian, Jean Pierre De; Perlman, Elizabeth J.; Trevenen, Cynthia; Rotta, Alexandre T.; Kalelkar, Mitra B.; Rowley, Anne H.

doi:10.1371/journal.pone.0038998

Cited by 313 publications

(357 citation statements)

References 61 publications

(69 reference statements)

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“…from autopsies of young adult KD patients has been demonstrated by immunohistochemistry [6]. Myofibroblasts in KD patients may remain activated and cause progressive luminal narrowing of coronary artery to the point of total occlusion [33]. In the present study, we demonstrated expression of Gal-3 in SMA-expressing spindle-shaped cells in myocardium.…”

Section: Discussionsupporting

confidence: 62%

Galectin-3 is a marker of myocardial and vascular fibrosis in Kawasaki disease patients with giant aneurysms

Numano

Shimizu

Jimenez-Fernandez

et al. 2015

International Journal of Cardiology

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Section: Discussionsupporting

confidence: 62%

Galectin-3 is a marker of myocardial and vascular fibrosis in Kawasaki disease patients with giant aneurysms

Numano

Shimizu

Jimenez-Fernandez

et al. 2015

International Journal of Cardiology

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“…73,131 Common sites include the axillary, subclavian, brachial, femoral, iliac, splanchnic, and mesenteric arteries, usually near or at branching points. These may present clinically as pulsatile masses and bruits.…”

Section: Other Arterial Abnormalitiesmentioning

confidence: 99%

“…Myocarditis in KD likely improves rapidly as the inflammatory process subsides because it results from interstitial edema and inflammation and only rarely from myocardial cell necrosis. 73,122 Infrequently, acute myocardial inflammation is associated with overt ventricular ectopy, although recent information indicates more common repolarization impact than may be clinically apparent (see Long-Term Management, Arrhythmias). The exception to the more typical short-term impact of mild myocarditis in KD is the KD shock syndrome.…”

mentioning

confidence: 99%

“…Indeed, most giant aneurysms seen at postmortem examination are lined by chronic thrombus. 73 Because both platelets and humoral clotting factors promote thrombus formation within giant aneurysms, patients are treated with a combination of antiplatelet and anticoagulant therapy, most commonly low-dose ASA together with either warfarin, maintaining an international normalized ratio of 2.0. to 3.0, or lowmolecular-weight heparin (LMWH). …”

mentioning

confidence: 99%

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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

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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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“…It was demonstrated that inflammatory cytokines are essential factors for the pathogenesis of inflammation in Kawasaki disease and that coronary artery lesions were caused by abnormal activation of endothelial cells, progressive injury of the arterial media and activation/ disruption of the coagulation and fibrinolysis systems [27,28].…”

Section: Plasma Exchangementioning

confidence: 99%

Pathogenesis of Systemic Inflammatory Diseases in Childhood: ?Lessons From Clinical Trials of Anti-Cytokine Monoclonal Antibodies for Kawasaki Disease, Systemic Onset Juvenile Idiopathic Arthritis, and Cryopyrin-Associated Periodic Fever Syndrome?

Yokota¹,

Kikuchi²,

Nozawa³

et al. 2013

Pediat Therapeut

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Inflammation has often been considered a non-specific response, and to play only a bridging role in the activation of adaptive immunity. However, it is now accepted that inflammation is the product of an independent innate immune system closely linked to the adaptive immune system. The key mediators of inflammation are inflammatory cytokines, as determined by multiple lines of evidence both in vitro and in vivo. Due to the crucial role of inflammatory cytokines in the pathogenesis of autoimmune disorders, anti-cytokine treatment has been developed as a therapy for rheumatoid arthritis, juvenile idiopathic arthritis (JIA), and inflammatory bowel disease. We recently completed several clinical trials of anti-cytokine treatment for children with systemic inflammatory diseases: anti-IL-6 receptor monoclonal antibody (tocilizumab) for children with 2 subtypes of JIA (poly-JIA and systemic JIA), anti-TNF-alpha monoclonal antibody (infliximab) for children with Kawasaki disease, and anti-IL-1-beta monoclonal antibody (canakinumab) for children with cryopyrin-associated periodic syndrome. This review summarizes the basis of inflammation in terms of innate immunity and adaptive immunity in these systemic inflammatory diseases, clinical efficacy and tolerability of these biologic agents, and attempts to determine the roles of individual inflammatory cytokines in disease pathogenesis.

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Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study

Cited by 313 publications

References 61 publications

Galectin-3 is a marker of myocardial and vascular fibrosis in Kawasaki disease patients with giant aneurysms

Galectin-3 is a marker of myocardial and vascular fibrosis in Kawasaki disease patients with giant aneurysms

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

Pathogenesis of Systemic Inflammatory Diseases in Childhood: ?Lessons From Clinical Trials of Anti-Cytokine Monoclonal Antibodies for Kawasaki Disease, Systemic Onset Juvenile Idiopathic Arthritis, and Cryopyrin-Associated Periodic Fever Syndrome?

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