Kawasaki disease in adults: Observations in France and literature review

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• 作者：Jean-Baptiste Fraison^a ; ^{jeanbaptiste.fraison@aphp.fr" class="auth_mail" title="E-mail the corresponding author} ; Pascal Sè ; ve^b ; Claire Dauphin^c ; Alfred Mahr^a ; Emeline Gomard-Mennesson^d ; Loig Varron^e ; Gregory Pugnet^f ; Cé ; dric Landron^g ; Pascal Roblot^g ; Eric Oziol^d ; Gihane Chalhoub^h ; Jean-Marc Galempoixⁱ ; Sé ; bastien Humbert^j ; Philippe Humbert^k ; Emilie Sbidian^l ; Florent Grange^m ; Olivier Bayrouⁿ ; Pascal Cathebras^o ; Philippe Morlat^p ; Olivier Epaulard^q ; Patricia Pavese^q ; Du Le Thi Huong^r ; Abdelkader Zoulim^s ; Katia Stankovic^t ; Hervé ; Bachelez^u ; Amar Smail^v ; C. Bachmeyer^w ; Brigitte Granel^x ; Jacques Serratrice^x ; Graziella Brinchault^y ; Arsè ; ne Mekinian^z ; Nathalie Costedoat-Chalumeau^aa ; Anne Bourgarit-Durand^ab ; Xavier Pué ; chal^aa ; Loï ; c Guillevin^aa ; Maryam Piram^ac ; Isabelle Koné ; -Paut^ac ; ¹ ; Olivier Fain^z ; ¹ ; on behalf of the CRI ; the French Vasculitis Study Group^aa
• 关键词：Mucocutaneous lymph node syndrome ; Adult ; Intravenous immunoglobulin
• 刊名：Autoimmunity Reviews
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：15
• 期：3
• 页码：242-249
• 全文大小：289 K

文摘

Kawasaki disease (KD) is a vasculitis that mostly occurs in young children and rarely in adults. We analyzed the characteristics of adult-onset KD (AKD) in France.

Methods

We collected retrospective and prospective data for patients with a diagnosis of KD occurring after the age of 18 years. Cases were obtained via various French medical networks and identified from the international literature.

Results

We included 43 patients of AKD at 26 institution from 1992 to 2015, with mean (SD) age 30 (11) years (range 18–68) and sex ratio (M/F) 1.2; 34 patients met the American Heart Association criteria and 9 were incomplete AKD. The median time to diagnosis was 13 days (interquartile range 8–21). The main symptoms were fever (100%), exanthema (98%), changes in the extremities (91%), conjunctivitis (77%), oral cavity changes (89%), cervical adenitis (55%) and cardiac abnormalities (45%). Overall, 35% of patients showed large-vessel vasculitis: coronary vasculitis (26%) and coronary aneurysm (19%). Treatment was mostly intravenous immunoglobulins (79%) and aspirin (81%). Four patients showed myocardial infarction due to coronary vasculitis, but none were treated with IVIg because of late diagnosis. After a median follow-up of 5 months (range 1–117), persistent aneurysm was noted in 9% of cases. Damage was significantly lower with early treatment than late or no treatment (p = 0.01).

Conclusion

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.