Université Paris Diderot, France
Jean-Baptiste Fraison has completed his Doctoral thesis in Internal Medicine in the year 2013 from Montpellier University School of Medicine. He is currently an
Assistant Professor in Internal Medicine department of Dr. Alfred Mahr (Hôpital St Louis, Université Paris Diderot, France). His research interests include vasculitis especially Behcet disease and Kawasaki disease.
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. 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 in 27 French centers. We included 44 cases of AKD, with mean (SD) age 31 (12) years (range 18–68) and sex ratio (M/F) 1:2; 33 cases met the American Heart Association criteria; 9 were incomplete and 2 were a typical AKD. The median time for diagnosis was 15 days (interquartile range 9–21). 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%). Biological findings revealed the elevated levels of acute-phase reactants (100%), hepatic cytolysis (64%) and elevated bilirubin level (23%). Overall, 32% of cases showed large-vessel vasculitis: Coronary vasculitis (25%), coronary aneurysm (16%) and peripheral vasculitis (4.5%). Treatment was mostly intravenous immunoglobulins (73%) and aspirin (81%). Out of all the cases, four cases 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 (interquartile 2-17, range 1–117), persistent aneurysm was noted in 7% of cases. No deaths were reported hence, damage was significantly lower with early treatment than late or no treatment (p=0.02).