Jean-Baptiste Fraison1, Pascal Sève2, Claire Dauphin3, Alfred Mahr4, Emeline Gomard-Mennesson5, Loig Varron6, Gregory Pugnet7, Cédric Landron8, Pascal Roblot8, Eric Oziol5, Gihane Chalhoub9, Jean-Marc Galempoix10, Sébastien Humbert11, Philippe Humbert12, Emilie Sbidian13, Florent Grange14, Olivier Bayrou15, Pascal Cathebras16, Philippe Morlat17, Olivier Epaulard18, Patricia Pavese18, Du Le Thi Huong19, Abdelkader Zoulim20, Katia Stankovic21, Hervé Bachelez22, Amar Smail23, C Bachmeyer24, Brigitte Granel25, Jacques Serratrice25, Graziella Brinchault26, Arsène Mekinian27, Nathalie Costedoat-Chalumeau28, Anne Bourgarit-Durand29, Xavier Puéchal28, Loïc Guillevin28, Maryam Piram30, Isabelle Koné-Paut30, Olivier Fain27. 1. Service de Médecine Interne, Hôpital Saint Louis, AP HP, Université Diderot, France. Electronic address: jeanbaptiste.fraison@aphp.fr. 2. Service de Médecine Interne, Hôpital de la Croix Rousse, Centre Hospitalier Universitaire de Lyon, Université de Lyon, France. 3. Service de Cardiologie, Hôpital Gabriel Montpied, Université de Clermont-Ferrand, France. 4. Service de Médecine Interne, Hôpital Saint Louis, AP HP, Université Diderot, France. 5. Service de Médecine Interne, Centre Hospitalier de Béziers, France. 6. Service de Médecine Interne, Centre Hospitalier de Montélimar, France. 7. Service de Médecine Interne, Centre Hospitalier Universitaire de Toulouse, France. 8. Service de Médecine Interne et Maladies Infectieuses, Centre Hospitalier Universitaire de Poitiers, France. 9. Service de Médecine Interne, Centre Hospitalier de Metz-Thionville, France. 10. Service de Médecine Interne, Centre Hospitalier de Charleville-Mézières, France. 11. Service de Médecine Interne, Centre Hospitalier Universitaire de Besançon, France. 12. Service de Dermatologie, Centre Hospitalier Universitaire de Besançon, University of Franche-Comté, INSERM UMR1098, SFR FED 4234 IBCT, Besançon, France. 13. Service de Dermatologie, Hôpital Henri Mondor, AP HP, Université Paris Est, France. 14. Service de Dermatologie, Centre Hospitalier Universitaire de Reims, France. 15. Service de Dermatologie, Hôpital Tenon, AP HP, Université Pierre et Marie Curie, France. 16. Service de Médecine Interne, Centre Hospitalier Universitaire de St Etienne, France. 17. Service de Médecine Interne et Maladies Infectieuses, Centre Hospitalier Universitaire de Bordeaux, France. 18. Service de Maladies Infectieuses, Centre Hospitalier Universitaire de Grenoble, France. 19. Service de Médecine Interne 2, Hôpital La Pitié-Salpétrière, AP HP, Université Pierre et Marie Curie, France. 20. Service de Médecine Interne, Centre Hospitalier Universitaire de Caen, France. 21. Service de Médecine Interne, Hôpital Tenon, AP HP, Université Pierre et Marie Curie, France. 22. Service de Dermatologie, Hôpital Saint Louis, AP HP, Université Diderot, France. 23. Service de Médecine Interne, Centre Hospitalier Universitaire d'Amiens, France. 24. Service de Médecine Interne, Centre Hospitalier de Creil, France. 25. Service de Médecine Interne, Hôpital Nord, AP HM, France. 26. Service de Pneumologie, Centre Hospitalier Universitaire de Rennes, France. 27. Service de Médecine Interne, DHUi2B, Hôpital Saint Antoine, AP HP, Université Pierre et Marie Curie, France. 28. Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques et Autoimmunes Rares, AP HP, Université Paris Descartes, France. 29. Service de Médecine Interne, Hôpital Jean Verdier, AP HP, Université Leonard de Vinci, France. 30. Service de Rhumatologie Pédiatrique, Centre de Référence des Maladies Auto-Inflammatoires de l'enfant, Hôpital Bicêtre, AP HP, Université Paris Sud, France.
Abstract
OBJECTIVE: 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.
OBJECTIVE: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.
Authors: Raymond M Johnson; Kelly R Bergmann; John J Manaloor; Xiaoqing Yu; James E Slaven; Anupam B Kharbanda Journal: Open Forum Infect Dis Date: 2016-09-29 Impact factor: 3.835
Authors: S M Dietz; D van Stijn; D Burgner; M Levin; I M Kuipers; B A Hutten; T W Kuijpers Journal: Eur J Pediatr Date: 2017-06-27 Impact factor: 3.183