Persistent Endothelial Damage After Intravenous Immunoglobulin Therapy In Kawasaki Disease

Sakurai, Yoshihiko; Takatsuka, Hideo; Takada, Mutsuzo; Nishino, Masami

doi:10.1016/j.jaci.2013.12.809

Cited by 4 publications

(10 citation statements)

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“…In a previous study of markers of endothelial damage in KD [59], my colleagues and I demonstrated that TM and AT levels are not elevated in the acute phase of KD. Thus, TM and AT levels cannot be used to adequately assess endothelial damage because of extravasation caused by enhanced vascular permeability [60,61] and because of increased renal excretion [62].…”

Section: Assessment Of Endothelial Damagementioning

confidence: 92%

“…In fact, thrombotic tendency is well recognized in the acute phase of KD and is associated with disseminated intravascular coagulation in some cases of KD [95][96][97][98]. In a previous study, my colleagues and I reported that D-dimer levels exceeded the reference value in the acute phase of KD, thus indicating thrombin generation in that phase [59].…”

Section: Autoimmunothrombosis In Kdmentioning

confidence: 99%

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Autoimmune Aspects of Kawasaki Disease

Sakurai¹

2019

J Investig Allergol Clin Immunol

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Kawasaki disease (KD) is classified as a medium-sized vasculitis of systemic vasculitis syndrome characterized by hypercytokinemia. Although the etiology of KD remains unidentified, epidemiological features point to the role of infection and genetic predisposition. Recent studies revealed endothelial damage and resultant thrombin generation, as well as B-cell activation during the acute phase of KD. Several anti-endothelial cell autoantibodies (AECAs) have been identified in KD patients. Taken together with the recently developed concept of immunothrombosis, a potential pathogenic mechanism for KD emerges. First, some polyclonal antibodies generated against invading microorganisms would exhibit cross-reactivity toward endothelial cell components and become dominant during affinity maturation. AECA binding to endothelial cells would cause endothelial activation or damage, with proinflammatory cytokine release, fostering a hypercoagulable state by leukocyte activation by proinflammatory cytokines. This, in turn, would lead to coronary artery lesions. KD vasculitis might be initiated upon AECA binding to the vasa vasorum and progress to panvasculitis and a vulnerable vessel wall, resulting in an aneurysm. The aneurysm would cause flow recirculation and alteration of wall shear stress. Consequently, platelets activated by shear stress, along with ultra-large von Willebrand factor (VWF) released by endothelial cells, would cause platelet-driven arterial thrombosis. Autoimmunity-associated thrombosis initiated by AECA binding to endothelial cells might play a major role in the pathogenesis of certain subtypes of KD. The notion of KD consisting of subtypes, the major one of which is AECA-associated vasculitis, will help facilitate a better understanding of KD and further promote early and accurate diagnosis, which remains challenging.

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Section: Assessment Of Endothelial Damagementioning

confidence: 92%

Section: Autoimmunothrombosis In Kdmentioning

confidence: 99%

Autoimmune Aspects of Kawasaki Disease

Sakurai¹

2019

J Investig Allergol Clin Immunol

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“…Therefore, the balance between coagulation and the fibrinolytic system may be disturbed during the acute stages of KD, particularly in patients with IVIG resistance, since KD vasculitis is accompanied by an increase in inflammatory cells and cytokines, and IVIG resistance may reflect a more severe inflammatory immune condition. Indeed, several studies have previously explored the coagulation profiles in patients with KD 7‐12 . However, most of them focused on the changes in coagulation profiles before and after IVIG in KD, 10 the role of coagulation profiles in distinguishing atypical KD from bacterial cervical lymphadenitis, 12 and in predicting the occurrence of CALs or cardiac complications 9‐11 .…”

Section: Introductionmentioning

confidence: 99%

Predictive value of coagulation profiles for both initial and repeated immunoglobulin resistance in Kawasaki disease: A prospective cohort study

Shao

Yang

Liu

et al. 2021

Pediatric Allergy Immunology

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Background: Intravenous immunoglobulin (IVIG) resistance prediction remains substantial in Kawasaki disease (KD), with limited data on the predictive value of coagulation profile for IVIG resistance, particularly for repeated IVIG resistance. Therefore, the aim of our study was to testify the predictive validity of coagulation profile for both initial IVIG resistance and repeated IVIG resistance in KD.Methods: A total of 385 KD patients were prospectively recruited between April 2015 and May 2019. Coagulation and other profiles were evaluated between the IVIG-responsive and IVIG-resistant groups. Multivariate logistic regression analysis was applied to determine the association between coagulation profiles and IVIG resistance. ROC curves analysis was further performed to assess the validity of coagulation profiles in predicting both initial IVIG resistance and repeated IVIG resistance.Results: Prothrombin time (PT), activated partial thromboplastin time (APTT), international normalized ratio (INR), fibrinogen degradation products (FDPs), and D-dimer were significantly increased in the initial IVIG-resistant group with antithrombin IIIThis is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

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“…The autoimmune-mediated hypercoagulable and prothrombotic mechanisms that predispose an individual of KD to cardiac complications are less well understood. A possible explanation is that the endothelial damage caused by the circulating antibodies compounded by the proinflammatory cytokines fosters the hypercoagulable state [27,28]. As the IVIg treatment ameliorates the inflammatory process, it is postulated to improve the hypercoagulable state manifested by the coagulation profile improvement [28,29].…”

Section: Discussionmentioning

confidence: 99%

Rethinking the Typical Diagnostic and Therapeutic Challenges of Kawasaki Disease

Mazahir

Saqqa²,

Magzoub³

et al. 2022

Dubai Med J

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This report presents a case of a 9-month-old male subject, with an initial presentation of a viral-like illness progressing rapidly to multiple deep neck abscesses involving the bilateral submandibular, parapharyngeal, retropharyngeal, and anterior mediastinal spaces subsequently confirmed with contrast-enhanced computed tomography of the neck. Additionally, the subject developed mucocutaneous signs of generalized erythematous rash, with red, fissured lips along with edema of the extremities. Initial lab parameters were suggestive of high inflammatory markers, low albumin, and deranged coagulation profile. Treatment with broad-spectrum intravenous antibiotics, albumin, and fresh frozen plasma infusions was commenced. Echocardiography revealed dilatation of the left main coronary artery with mild mitral valvular regurgitation. Based on the clinical criteria, a diagnosis of typical Kawasaki disease (KD) on day 5 of illness was made, and the subject received intravenous immunoglobulin (IVIg) and aspirin. The clinical course was complicated by IVIg resistance, for which a second dose of IVIg and pulse systemic corticosteroid therapy was required. Post defervescence, an isolated elevation of activated thromboplastin time, partially corrected with mixing studies and a positive circulating lupus anticoagulant was noted. The patient did not develop any bleeding or thromboembolic complications during his hospital stay. Surgical drainage of the neck abscesses did not yield any pus or fluid collection. After a stormy course of 31 days, the subject was discharged with an improving trend of inflammatory markers and no progression of coronary artery dilatation. Gradual resolution to baseline health was noted upon follow-up, 18 months postdischarge from the hospital. Despite being one of the most common cause of childhood acquired cardiac disease, KD still poses a significant diagnostic and treatment challenge to physicians. This requires physicians to be vigilant for timely diagnosis and appropriate management to prevent significant cardiac comorbidities and be aware of the possible complications that might arise during the treatment.

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Persistent Endothelial Damage After Intravenous Immunoglobulin Therapy In Kawasaki Disease

Cited by 4 publications

References 0 publications

Autoimmune Aspects of Kawasaki Disease

Autoimmune Aspects of Kawasaki Disease

Predictive value of coagulation profiles for both initial and repeated immunoglobulin resistance in Kawasaki disease: A prospective cohort study

Rethinking the Typical Diagnostic and Therapeutic Challenges of Kawasaki Disease

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