Kawasaki disease in adults: Observations in France and literature review

Fraison, J.B.; Sève, P.; Dauphin, Claire; Mahr, Alfred; Gomard-Mennesson, E.; Varron, L.; Pugnet, G.; Landron, C.; Roblot, P.; Oziol, E.; Chalhoub, G.; Galempoix, J.M.; Humbert, S.; Humbert, Philippe; Sbidian, É.; Grange, Florent; Bayrou, O.; Cathébras, P.; Morlat, Philippe; Épaulard, Olivier; Pavèse, Patricia; Huong, D. Lê Thi; Zoulim, A.; Stankovic, K.; Bachelez, Hervé; Смаил, А.; Bachmeyer, Claude; Granel, B.; Serratrice, Jacques; Brinchault, Graziella; Mékinian, A.; Costédoat-Chalumeau, Nathalie; Bourgarit-Durand, A.; Puéchal, Xavier; Guillevin, Loı̈c; Piram, Maryam; Koné‐Paut, Isabelle; Fain, Olivier

doi:10.1016/j.autrev.2015.11.010

Cited by 57 publications

(52 citation statements)

References 90 publications

(59 reference statements)

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“…The highest incidence is found in Japan; the most recent nationwide survey reported an incidence of 265/100,000 children under the age of 5 in 2012, and suggested that the incidence of KD is still rising [78]. Most patients are 6 months to 5 years old, although cases in older children and adults also occur [36]. The male/female ratio is approximately 1.5 to 1 [120].…”

Section: Epidemiologymentioning

confidence: 99%

Dissecting Kawasaki disease: a state-of-the-art review

et al. 2017

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Kawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. The diagnosis is based on the presence of persistent fever and clinical features including exanthema, lymphadenopathy, conjunctival injection, and changes to the mucosae and extremities. Although the etiology remains unknown, the current consensus is that it is likely caused by an (infectious) trigger initiating an abnormal immune response in genetically predisposed children. Treatment consists of high dose intravenous immunoglobulin (IVIG) and is directed at preventing the development of CAA. Unfortunately, 10–20% of all patients fail to respond to IVIG and these children need additional anti-inflammatory treatment. Coronary artery lesions are diagnosed by echocardiography in the acute and subacute phases. Both absolute arterial diameters and z-scores, adjusted for height and weight, are used as criteria for CAA. Close monitoring of CAA is important as ischemic symptoms or myocardial infarction due to thrombosis or stenosis can occur. These complications are most likely to arise in the largest, so-called giant CAA. Apart from the presence of CAA, it is unclear whether KD causes an increased cardiovascular risk due to the vasculitis itself. Conclusion: Many aspects of KD remain unknown, although there is growing knowledge on the etiology, treatment, and development and classification of CAA. Since children with previous KD are entering adulthood, long-term follow-up is increasingly important. What is known: • Kawasaki disease (KD) is a pediatric vasculitis with coronary artery damage as its main complication. • Although KD approaches its 50th birthday since its first description, many aspects of the disease remain poorly understood. What is new: • In recent years, multiple genetic candidate pathways involved in KD have been identified, with recently promising information about the ITPKC pathway. • As increasing numbers of KD patients are reaching adulthood, increasing information is available about the long-term consequences of coronary artery damage and broader cardiovascular risk.

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Section: Epidemiologymentioning

confidence: 99%

Dissecting Kawasaki disease: a state-of-the-art review

et al. 2017

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“…Treatment with intravenous immunoglobulin reduces the risk of coronary aneurysms in childhood Kawasaki disease from 30% to 3% 7 . While Fraison and colleagues 5 found that early intravenous immunoglobulin treatment reduced the duration of fever, it did not significantly reduce the number of cardiac complications. A larger study assessing the role of intravenous immunoglobulin in adult‐onset Kawasaki disease is needed.…”

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confidence: 93%

“…The largest study of adult‐onset Kawasaki disease, by Fraison and colleagues, 5 reviewed 43 cases in France and found that, compared with childhood Kawasaki disease, adults are more likely to have liver function anomalies and arthralgias, and are less likely to have thrombocytosis, cheilitis and meningitis 3 , 5 . Coronary artery aneurysms are a devastating complication of this disease, and the rate of coronary complications in both adult‐onset and childhood Kawasaki disease is similar 5 …”

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confidence: 99%

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A case of adult‐onset Kawasaki disease

Gehrmann

Morwood

Gillis

et al. 2018

Medical Journal of Australia

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“…However, frequency of clinical signs may vary between the two populations. [61] Although rare, adult onset KD is associated with high frequency of cardiovascular involvement. Because of its rarity and need to exclude other differential diagnoses, adult onset KD is usually diagnosed late.…”

Section: Introductionmentioning

confidence: 99%

Kawasaki disease for dermatologists

Gupta

Singh

2016

Indian Dermatol Online J

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Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. There is no specific laboratory test for KD. Diagnosis is clinical and depends upon the presence of fever for ≥5 days and 4 or more of five principal features, viz. polymorphous exanthem, extremity changes, mucosal changes involving the lips and oral cavity, bilateral bulbar conjunctival injection, and unilateral cervical lymphadenopathy. The term “incomplete KD” refers to the presence of fever and less than four principal clinical features. Recognition of this group of patients is important because it is usually seen in infants and risk of coronary abnormalities is increased probably because of delays in diagnosis. However, what appears to be “incomplete” at a given point of time may not actually be so because some of the features may have already subsided and others may evolve over time. Hence, a detailed dermatological examination is warranted in all cases, especially in incomplete KD, to ensure timely diagnosis. Although KD is a self-limiting disease in most patients, coronary artery abnormalities (CAAs) including coronary dilatations and aneurysms may develop in up to 25% of untreated patients. CAAs are the most common cause of morbidity and mortality in patients with KD. Treatment is aimed at reducing inflammation and consists of intravenous immunoglobulin (IVIG) along with aspirin. Despite treatment, some patients may still develop CAAs, and hence, long-term follow up is of utmost importance.

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Kawasaki disease in adults: Observations in France and literature review

Abstract: Given the high frequency of cardiac involvement and complications in this series of AKD, diagnosis and treatment should not be delayed, and early IVIg treatment seems to improve the outcome.

Cited by 57 publications

References 90 publications

Dissecting Kawasaki disease: a state-of-the-art review

Dissecting Kawasaki disease: a state-of-the-art review

A case of adult‐onset Kawasaki disease

Kawasaki disease for dermatologists

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