Sequelae of Kawasaki disease in adolescents and young adults

Burns, Jane C.; Shike, Hiroko; Gordon, John B.; Malhotra, Alka; Schoenwetter, Melissa; Kawasaki, Tomisaku

doi:10.1016/0735-1097(96)00099-x

Cited by 297 publications

(220 citation statements)

References 36 publications

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“…221,222 A history of a Kawasaki diseaselike illness in childhood should be sought in patients who present with coronary aneurysms in the absence of generalized atherosclerotic disease. Some adult patients may be unable to recall an illness that occurred so early in life, however.…”

Section: Course Of Patients With Persistent Coronary Artery Abnormalimentioning

confidence: 99%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease

Newburger¹,

Takahashi²,

Gerber³

et al. 2004

Circulation

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1,613

818

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Background— Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ∼15% to 25% of untreated children and may lead to ischemic heart disease or sudden death. Methods and Results— A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for ≥5 days and ≤4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-α antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata. Conclusions— Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.

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Section: Course Of Patients With Persistent Coronary Artery Abnormalimentioning

confidence: 99%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease

Newburger¹,

Takahashi²,

Gerber³

et al. 2004

Circulation

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1,613

818

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“…The diagnosis of KD is dependent upon clinical manifestations; definitive diagnostic markers are lacking. The pathogenesis of KD is characterized by immune injury to endothelial cells (1) that results in systemic vasculitis or aneurysms in coronary arteries (2).…”

mentioning

confidence: 99%

Early decreased plasma levels of factor B and C5a are important biomarkers in children with Kawasaki disease

Zou

Song

et al. 2015

Pediatr Res

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Background:The mechanisms underpinning Kawasaki disease (KD) are incompletely understood. There is an unmet need for specific biomarkers for the early diagnosis of KD. Methods: Eighty-five KD patients suffering from acute-phase and subacute-phase KD, 40 healthy children, and 40 febrile children comprised the study cohort. An enzyme-linked immunosorbent assay was used to measure plasma levels of C1q, C1q-circulating immune complex (C1q-CIC), mannan-binding lectin-associated serine protease (MASP)-1, factor B, C4d, C3d, C5a, C5b-9 and CD59. results: Plasma concentrations of factor B and C5a in the acute phase were lower than those in healthy and febrile control groups (all P < 0.05). Compared with acute-phase KD patients, plasma concentrations of C1q, factor B, and C3d in KD patients were increased significantly (P < 0.05), but those of C4d, MASP-1 and CD59 decreased significantly (P < 0.05), in patients with sub-acute KD. conclusion: These data suggest that more than one pathway in the complement system is activated in KD. Importantly, decreased plasma concentrations of factor B and C5a in the acute phase (6-10 d) could be employed as biomarkers for the early diagnosis of KD. k awasaki disease (KD) is an acute, self-limiting, nonspecific systemic vascular inflammatory syndrome. KD is considered to be caused by infectious agents in genetically susceptible children. The diagnosis of KD is dependent upon clinical manifestations; definitive diagnostic markers are lacking. The pathogenesis of KD is characterized by immune injury to endothelial cells (1) that results in systemic vasculitis or aneurysms in coronary arteries (2).Vascular endothelial cells are a source of complement and complement regulatory factors. Activated complement can form complement fragments (e.g., C3, C4d, Bb) and the membrane attack complex (MAC) on the cell surface (3). The MAC can mediate the inflammatory response by killing anucleate cells and bacteria, as well as causing cells to secrete a large number of cytokines and growth factors (4). Animal studies have shown that coronary atherosclerotic lesions appear not only during complement activation, but also during complement deposition, which results in tissue injury (5). It also has been demonstrated that complement components are accumulated in cryoglobulinemic vasculitis (6). Other studies have reported dysfunction of vascular endothelial cells (7) and the classical pathway of complement activation in all three layers of aneurysms (8).With the evidence stated above, we speculated that activation of the complement system might be involved in the pathogenesis of KD, and that activated complement factors could be employed as early diagnostic markers for KD. To test this hypothesis, we investigated plasma levels of various complement components in children with acute KD and subacute KD. RESULTS Changes in Plasma Concentrations of Complement FactorsC1q and C1q-circulating immune complex (C1q-CIC). C1q is a factor of the classical pathway of complement activation. The plasma level of C1q was sig...

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“…K awasaki Disease (KD) 3 is an acute vasculitis of young childhood which may be fatal. KD is the leading cause of acquired heart disease in children in developed nations (1), but little is known about the pathogenesis of the disorder.…”

mentioning

confidence: 99%

“…The symptoms of KD resolve over several weeks, even in untreated patients, and recurrences are unusual, distinguishing the illness from autoimmune vasculitic disorders. The sequelae of undiagnosed KD during childhood appears to account for cases of acute myocardial infarction or sudden death in young adults (3). Because the etiology of KD is unknown, no diagnostic test is available, specific therapy cannot be developed, and prevention is not feasible.…”

mentioning

confidence: 99%

Oligoclonal IgA Response in the Vascular Wall in Acute Kawasaki Disease

Rowley

Shulman

Spike

et al. 2001

The Journal of Immunology

182

113

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Kawasaki Disease (KD) is a potentially fatal acute vasculitis of childhood. Although KD is the leading cause of acquired heart disease in children in developed nations, its pathogenesis remains unknown. We previously reported the novel observation that IgA plasma cells infiltrate the vascular wall in acute KD. We have now examined the clonality of this IgA response in vascular tissue from three fatal cases of KD to determine whether it is oligoclonal, suggesting an Ag-driven process, or polyclonal, suggesting nonspecific B cell activation or a response to a superantigen. We first sequenced VDJ junctions of 44 α genes isolated from a primary, unamplified KD vascular cDNA library. Five sets of clonally related α sequences were identified, comprising 34% (15 of 44) of the isolated α sequences. Furthermore, point mutations consistent with somatic mutation were detected in the related sequences. Next, using formalin-fixed coronary arteries from two additional fatal KD cases, we sequenced VDJ junctions of α genes isolated by RT-PCR, and a restricted pattern of CDR3 usage was observed in both. We conclude that the vascular IgA response in acute KD is oligoclonal. The identification of an oligoclonal IgA response in KD strongly suggests that the immune response to this important childhood illness is Ag-driven.

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Sequelae of Kawasaki disease in adolescents and young adults

Cited by 297 publications

References 36 publications

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease

Early decreased plasma levels of factor B and C5a are important biomarkers in children with Kawasaki disease

Oligoclonal IgA Response in the Vascular Wall in Acute Kawasaki Disease

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