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Concept: Kawasaki disease

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Kawasaki disease (KD) is a systemic vasculitis and affects many organ systems. It often presents sterile pyuria, microscopic hematuria, and proteinuria due to renal involvement. The aims of this study were to define clinical characteristics of acute KD patients with pyuria and to analyze meaning of pyuria in KD.

Concepts: Nephrology, Vasculitis, Nephrotic syndrome, Arthralgia, Glomerulonephritis, Kawasaki disease, Urinalysis, IgA nephropathy

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Kawasaki disease (KD) is the most common pediatric vasculitis. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients.

Concepts: Immune system, Antibody, Vasculitis, Wegener's granulomatosis, Intravenous immunoglobulin, X-linked agammaglobulinemia, Kawasaki disease

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An 8-year-old girl was admitted to the hospital with a 7-day history of fever and a diffuse macular rash. She had fissured lips and strawberry tongue, and her temperature was 39°C. A clinical diagnosis of Kawasaki’s disease was made.

Concepts: Cardiology, Aspirin, Kawasaki disease, Rash, Scarlet fever, Strawberry tongue

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Objective: Current guidelines for Kawasaki disease (KD) recommend intravenous immunoglobulin infusion and echocardiography. However, no previous studies have evaluated the relationship between the treating hospital’s concordance with guidelines and the prevention of coronary artery aneurysm in patients with KD. Study Design: KD patients between 2010 and 2013 were identified in a Japanese national inpatient database. Guideline concordance was defined as the proportions of patients who received echocardiography as well as treatment with both immunoglobulin and aspirin, which were divided into quartiles. Multivariable logistic regression analysis was conducted to examine the association between the guideline concordance and the occurrence of coronary artery aneurysm with adjustment for patient backgrounds. Results: In sum, 20 156 patients with KD were identified. The very high (>83.3%) concordance group had a lower rate of coronary artery aneurysm than the very low (<59.0%) concordance group (4.9% vs 9.9%; odds ratio, 0.45; 95% CI, 0.27 to 0.74; P = .002). Conclusion: All patients with KD should be treated according to the guidelines.

Concepts: Regression analysis, Logistic regression, Atherosclerosis, Cardiology, Artery, Intravenous immunoglobulin, Kawasaki disease, Coronary artery aneurysm

23

Understanding global seasonal patterns of Kawasaki disease (KD) may provide insight into the etiology of this vasculitis that is now the most common cause of acquired heart disease in children in developed countries worldwide.

Concepts: Heart, Vasculitis, Developed country, Heart disease, Kawasaki disease, Canada

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Background: The occurrence of a distinctive perineal eruption that appears early in infants with Kawasaki disease (KD), the most relevant type of febrile vasculitis of childhood, has received little attention in pediatric reports. KD diagnosis is based on clinical criteria, which can be supported by laboratory abnormalities or positive echocardiography findings: difficulty in diagnosis can be increased by incomplete or atypical presentations, but a timely diagnostic process is essential in the youngest patients who are more prone to the risk of cardiac sequelae resulting from KD. Case Presentation: In this report, we present the case of a 2-month-old infant with an unusual presentation of KD, in whom diagnosis was made despite fever remission on the fourth day of hospitalization following intravenous corticosteroid therapy to treat concomitant bronchoconstriction. The presence of early desquamating perineal erythema led to the consideration of KD diagnosis, confirmed by the echocardiographic assessment of right and left coronary artery dilatations with pericardial effusion on the fifth day of hospital stay. Conclusions: Diagnosis of KD represents a demanding challenge, mainly when the illness starts with an incomplete or nuanced presentation. An erythematous desquamating perineal rash is a valuable early clinical clue, which might facilitate a prompt diagnosis of KD. This case emphasizes that an accurate assessment of all clinical features, including perineal erythema with early tendency to desquamation, and an eventual echocardiography, are necessary in an infant displaying fever to corroborate the suspicion of KD.

Concepts: Cardiology, Heart, Artery, Coronary circulation, Left coronary artery, Aspirin, Pediatrics, Kawasaki disease

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Kawasaki disease (KD) is an acute self-limiting inflammatory disorder, associated with vasculitis, affecting predominantly medium-sized arteries, particularly the coronary arteries. In developed countries KD is the commonest cause of acquired heart disease in childhood. The aetiology of KD remains unknown, and it is currently believed that one or more as yet unidentified infectious agents induce an intense inflammatory host response in genetically susceptible individuals. Genetic studies have identified several susceptibility genes for KD and its sequelae in different ethnic populations, including FCGR2A, CD40, ITPKC, FAM167A-BLK and CASP3, as well as genes influencing response to intravenous immunoglobulin (IVIG) and aneurysm formation such as FCGR3B, and transforming growth factor (TGF) β pathway genes. IVIG and aspirin are effective therapeutically, but recent clinical trials and meta-analyses have demonstrated that the addition of corticosteroids to IVIG is beneficial for the prevention of coronary artery aneurysms (CAA) in severe cases with highest risk of IVIG resistance. Outside of Japan, however, clinical scores to predict IVIG resistance perform suboptimally. Furthermore, the evidence base does not provide clear guidance on which corticosteroid regimen is most effective. Other therapies, including anti-TNFα, could also have a role for IVIG-resistant KD. Irrespective of these caveats, it is clear that therapy that reduces inflammation in acute KD, improves outcome. This paper summarises recent advances in the understanding of KD pathogenesis and therapeutics, and provides an approach for managing KD patients in the UK in the light of these advances.

Concepts: Immune system, Atherosclerosis, Heart, Atheroma, Artery, Coronary circulation, Intravenous immunoglobulin, Kawasaki disease

5

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.

Concepts: Atherosclerosis, Heart, Stroke, Vasculitis, Artery, Coronary circulation, Kawasaki disease, Coronary artery aneurysm

4

Resistance to intravenous immunoglobulin (IVIG) occurs in 10-20% of patients with Kawasaki disease (KD). The risk of resistance is about two-fold higher in patients with elevated gamma glutamyl transferase (GGT) levels. We sought to understand the biological mechanisms underlying IVIG resistance in patients with elevated GGT levels.

Concepts: Immune system, Antibody, Glycoproteins, Molecular biology, Intravenous immunoglobulin, X-linked agammaglobulinemia, Medical treatments, Kawasaki disease

3

The timing and selection of patients with Kawasaki disease for corticosteroid use to prevent coronary artery complications remain controversial.

Concepts: Artery, Coronary circulation, Kawasaki disease