How Should We Classify Kawasaki Disease?

Marrani, Edoardo; Burns, Jane C.; Cimaz, Rolando

doi:10.3389/fimmu.2018.02974

Cited by 53 publications

(56 citation statements)

References 62 publications

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“…Kawasaki disease (KD) is the leading cause of acquired pediatric heart disease in the developed world, presenting in young children as acute, febrile, self-limiting, systemic vasculitis ( 1 , 2 ). The clinical and epidemiological features of KD have suggested an infectious cause ( 3 ). However, the etiology of the disease remains unknown.…”

Section: Introductionmentioning

confidence: 99%

“…Current theories of KD pathogenesis include (i) infection with classic immune responses to an as-yet identified pathogen(s), (ii) an autoantibody or T-cell driven autoimmune response triggered by an antigen, i.e., molecular mimicry, and (iii) an autoinflammatory response, which includes innate immune responses that cause systemic inflammation as well as damage to the coronary arterial wall ( 3 ). Previous studies have focused on cytokines and chemokines during acute KD.…”

Section: Introductionmentioning

confidence: 99%

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Elevated Levels of Pentraxin 3 Correlate With Neutrophilia and Coronary Artery Dilation During Acute Kawasaki Disease

et al. 2020

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Kawasaki disease (KD) is the leading cause of acquired pediatric heart disease in the developed world as 25–30% of untreated patients and at least 5% of treated patients will develop irreversible coronary artery lesions (CAL). Pentraxin-3 (PTX-3) has been well-studied in inflammatory diseases, particularly in cardiovascular diseases associated with vascular endothelial dysfunction. We hypothesized that PTX-3 plays an important role in the development of KD-associated CAL and investigated the circulating levels of PTX-3 in the serum of KD patients. Children with acute KD were followed from diagnosis through normalization of the clinical parameters of inflammation (convalescent phase). Serum samples were obtained and echocardiograms were conducted at several phases of the illness: acute [prior to intravenous immunoglobulin (IVIG) treatment], sub-acute (5–10 days after IVIG treatment), and convalescent (1–4 months after KD diagnosis). Seventy children were included in the final cohort of the study, of whom 26 (37%) presented with CAL and 18 (26%) developed IVIG resistance. The patients included in this study came from diverse ethnic backgrounds, mostly with mixed ancestry/ ethnicity. Significantly increased PTX-3 levels were observed during the acute phase of KD compared to the sub-acute and the convalescent phases. The PTX-3 levels during acute KD were significantly higher among KD patients with CAL compared to patients with normal coronary arteries (NCA). Also, the PTX-3 levels were significantly higher in patients with IVIG resistance. Furthermore, the PTX-3 levels were significantly higher in IVIG-resistant KD patients with CAL as compared to the NCA group. Moreover, the PTX-3 levels were significantly correlated to coronary artery z-score during acute KD and to neutrophil counts throughout KD progression regardless of coronary artery z-score. Elevated PTX-3 levels correlated to elevated neutrophil counts, a known source of PTX-3 in acute inflammation and an important player in the development of KD vasculitis. We, therefore, suggest PTX-3 as a novel factor in the development of KD-associated CAL and propose neutrophil-derived PTX-3 as contributing to KD vascular dysfunction.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Elevated Levels of Pentraxin 3 Correlate With Neutrophilia and Coronary Artery Dilation During Acute Kawasaki Disease

et al. 2020

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“…The 10-, 20-, 30-and 35-year risks of being diagnosed with autoimmune diseases after the primary KD diagnosis were all significantly increased ( Table 3). The median time to autoimmune disease was 2 years (Q1-Q3 [0-12]) in the KD group and 12 years (Q1-Q3 [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]) in the control group. The median follow-up time at risk was 11 years in the KD group and 12 years in the control group.…”

Section: Autoimmune Disease and Cancermentioning

confidence: 99%

Kawasaki disease, autoimmune disorders, and cancer: a register-based study

et al. 2020

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Kawasaki disease has well-described cardiovascular complications. However, the association to autoimmunity and cancer in the long term is not well described. We investigated theses associations using a registry-based matched cohort follow-up study of patients diagnosed with Kawasaki disease. Patients with Kawasaki disease were included and matched 1:5 to a population control group, matched by birth year, sex and incident month of the Kawasaki disease diagnosis. A total of 820 cases < 21 years of age were identified. Median age at diagnosis was 3 years. Median follow-up time was 12 years. Patients with KD were at higher risk of being diagnosed with ischaemic heart disease at 10 years (HR 39.94 (95% CI 5.00-319.28)) and 30 years (HR 8.33 (95% CI 3.03-22.91)). The 10-, 20-and 30-year risks of developing autoimmune disorders were HR 4.23 (95% CI 3.01-5.94), HR 3.23 (95% CI 2.44-4.29) and 2.83 (95% CI, 2.17-3.68), all p < 0.001. Cancer risk was increased after 30 years (HR 2.42 (95% CI, 1.09-5.34)). All-cause mortality after 35 years was also significantly increased (HR 3.14 (95% CI, 1.03-9.60)). Children with KD have increased long-term risks of ischaemic heart disease also of autoimmune disease and cancer, as well as an increased allcause mortality. The surprisingly increased risk of autoimmunity must be investigated further. What is known: • Kawasaki disease is characterized by acute vasculitis and inflammation that can affect the coronary arteries. • Anti-inflammatory medicine is effective in the acute stages of the disease. What is new: • Children with Kawasaki disease have an increased risk of developing autoimmune disease in the long term. • Kawasaki disease is associated with a slightly increased mortality rate driven by non-cardiovascular causes.

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“…More recently, a rare pediatric syndrome associated with COVID-19 disease, and similar to Kawasaki Disease, has been described and is characterized by systemic inflammation and organ dysfunction (Han and Lee 2018;Nathan et al 2020). The vascular injury described in Kawasaki disease is associated with coronary artery aneurysms and is direct evidence of vascular injury (Marrani et al 2018).…”

mentioning

confidence: 99%