title: Kawasaki disease in adults: Observations in France and literature review.
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).
  
  
  CONCLUSIONS
  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.affiliation: Service de Médecine Interne, Hôpital Saint Louis, AP HP, Université Diderot, France. Electronic address: jeanbaptiste.fraison@aphp.fr.
authors.forename: Jean-Baptiste
authors.initials: JB
authors.lastname: Fraison
authors.affiliation: Service de Médecine Interne, Hôpital de la Croix Rousse, Centre Hospitalier Universitaire de Lyon, Université de Lyon, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Sève
authors.affiliation: Service de Cardiologie, Hôpital Gabriel Montpied, Université de Clermont-Ferrand, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Dauphin
authors.affiliation: Service de Médecine Interne, Hôpital Saint Louis, AP HP, Université Diderot, France.
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.affiliation: Service de Médecine Interne, Centre Hospitalier de Béziers, France.
authors.forename: Emeline
authors.initials: E
authors.lastname: Gomard-Mennesson
authors.affiliation: Service de Médecine Interne, Centre Hospitalier de Montélimar, France.
authors.forename: Loig
authors.initials: L
authors.lastname: Varron
authors.affiliation: Service de Médecine Interne, Centre Hospitalier Universitaire de Toulouse, France.
authors.forename: Gregory
authors.initials: G
authors.lastname: Pugnet
authors.affiliation: Service de Médecine Interne et Maladies Infectieuses, Centre Hospitalier Universitaire de Poitiers, France.
authors.forename: Cédric
authors.initials: C
authors.lastname: Landron
authors.affiliation: Service de Médecine Interne et Maladies Infectieuses, Centre Hospitalier Universitaire de Poitiers, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Roblot
authors.affiliation: Service de Médecine Interne, Centre Hospitalier de Béziers, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Oziol
authors.affiliation: Service de Médecine Interne, Centre Hospitalier de Metz-Thionville, France.
authors.forename: Gihane
authors.initials: G
authors.lastname: Chalhoub
authors.affiliation: Service de Médecine Interne, Centre Hospitalier de Charleville-Mézières, France.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Galempoix
authors.affiliation: Service de Médecine Interne, Centre Hospitalier Universitaire de Besançon, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Humbert
authors.affiliation: Service de Dermatologie, Centre Hospitalier Universitaire de Besançon, University of Franche-Comté, INSERM UMR1098, SFR FED 4234 IBCT, Besançon, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Humbert
authors.affiliation: Service de Dermatologie, Hôpital Henri Mondor, AP HP, Université Paris Est, France.
authors.forename: Emilie
authors.initials: E
authors.lastname: Sbidian
authors.affiliation: Service de Dermatologie, Centre Hospitalier Universitaire de Reims, France.
authors.forename: Florent
authors.initials: F
authors.lastname: Grange
authors.affiliation: Service de Dermatologie, Hôpital Tenon, AP HP, Université Pierre et Marie Curie, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Bayrou
authors.affiliation: Service de Médecine Interne, Centre Hospitalier Universitaire de St Etienne, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cathebras
authors.affiliation: Service de Médecine Interne et Maladies Infectieuses, Centre Hospitalier Universitaire de Bordeaux, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Morlat
authors.affiliation: Service de Maladies Infectieuses, Centre Hospitalier Universitaire de Grenoble, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Epaulard
authors.affiliation: Service de Maladies Infectieuses, Centre Hospitalier Universitaire de Grenoble, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Pavese
authors.affiliation: Service de Médecine Interne 2, Hôpital La Pitié-Salpétrière, AP HP, Université Pierre et Marie Curie, France.
authors.forename: Du Le Thi
authors.initials: du LT
authors.lastname: Huong
authors.affiliation: Service de Médecine Interne, Centre Hospitalier Universitaire de Caen, France.
authors.forename: Abdelkader
authors.initials: A
authors.lastname: Zoulim
authors.affiliation: Service de Médecine Interne, Hôpital Tenon, AP HP, Université Pierre et Marie Curie, France.
authors.forename: Katia
authors.initials: K
authors.lastname: Stankovic
authors.affiliation: Service de Dermatologie, Hôpital Saint Louis, AP HP, Université Diderot, France.
authors.forename: Hervé
authors.initials: H
authors.lastname: Bachelez
authors.affiliation: Service de Médecine Interne, Centre Hospitalier Universitaire d'Amiens, France.
authors.forename: Amar
authors.initials: A
authors.lastname: Smail
authors.affiliation: Service de Médecine Interne, Centre Hospitalier de Creil, France.
authors.forename: C
authors.initials: C
authors.lastname: Bachmeyer
authors.affiliation: Service de Médecine Interne, Hôpital Nord, AP HM, France.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Granel
authors.affiliation: Service de Médecine Interne, Hôpital Nord, AP HM, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Serratrice
authors.affiliation: Service de Pneumologie, Centre Hospitalier Universitaire de Rennes, France.
authors.forename: Graziella
authors.initials: G
authors.lastname: Brinchault
authors.affiliation: Service de Médecine Interne, DHUi2B, Hôpital Saint Antoine, AP HP, Université Pierre et Marie Curie, France.
authors.forename: Arsène
authors.initials: A
authors.lastname: Mekinian
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques et Autoimmunes Rares, AP HP, Université Paris Descartes, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.affiliation: Service de Médecine Interne, Hôpital Jean Verdier, AP HP, Université Leonard de Vinci, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Bourgarit-Durand
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques et Autoimmunes Rares, AP HP, Université Paris Descartes, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques et Autoimmunes Rares, AP HP, Université Paris Descartes, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.affiliation: Service de Rhumatologie Pédiatrique, Centre de Référence des Maladies Auto-Inflammatoires de l'enfant, Hôpital Bicêtre, AP HP, Université Paris Sud, France.
authors.forename: Maryam
authors.initials: M
authors.lastname: Piram
authors.affiliation: Service de Rhumatologie Pédiatrique, Centre de Référence des Maladies Auto-Inflammatoires de l'enfant, Hôpital Bicêtre, AP HP, Université Paris Sud, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Koné-Paut
authors.affiliation: Service de Médecine Interne, DHUi2B, Hôpital Saint Antoine, AP HP, Université Pierre et Marie Curie, France.
authors.forename: Olivier
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mesh_terms: D000328:Adult; D001241:Aspirin / Q000627:therapeutic use; D002318:Cardiovascular Diseases / Q000209:etiology; D005602:France; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications / Q000628:therapy; D014481:United States
chemical_list: D016756:Immunoglobulins, Intravenous; D001241:Aspirin
keywords: Adult; Intravenous immunoglobulin; Mucocutaneous lymph node syndrome