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French observatory of Kawasaki disease in adults: 24 observations

CIRCULATION(2015)

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Abstract
Introduction: Kawasaki disease (KD) is a vasculitis that occurs mostly among children and exceptionally in adults. We report data from a French observatory of adult KD. Patients and methods: Adult patients diagnosed with KD in 16 French centers were included. Patients were classified as complete KD or incomplete KD according to IKDC or probable KD. Results: We included 24 patients with a median age of 29 years (22-39) and a sex ratio (M / F) 2.42, 12 complete KD, 9 incomplete KD and 3 probable KD without any other cause. Time to diagnosis was 13 days (10-20.5). Main events were: fever (100%), extremities changes (21/24, 87.5%), rash (22/24, 92%), conjunctivitis (16/24, 16, 66%), cheilitis (15/24, 63%), strawberry tongue (11/24, 46%), adenopathy (10/24, 42%), cardiac abnormalities (11/24, 46%), cardiogenic shock (n = 1), myo-pericarditis (n = 3) and left heart failure (n = 1). Median CRP was 228mg/L (166-311), SGOT: 68 IU/L (51-139), SGPT: 125 IU/L (69-190), platelets 372 G/L (209-630) and leukocytes 16 G/L (8.3-20). Cardiac involvement was researched in 23 patients (96%) by achieving: echocardiography (20/24), coronary scanners (6/24), coronary angiography (5/24), cardiac MRI (2/24) and stress tests (2/24). An arteritic vascular disease was found in 11 patients (46%): coronary aneurysms (8/24, 33%), coronary arteritis (10/24; 42%) and peripheral arteritis (2/24, 8.3%) with acute lower limb ischemia (1/24, 4.2%) and splenic infarction (1/24, 4.2%). Patients received: intravenous immunoglobulin (17/24; 71%): aspirin (21/24, 88%). After 6 months, it persisted 5 aneurysm (20.8%). Complications noticed during the last follow-up were: heart failure (1/24, 4.3%), aneurysm (3/24, 12.5%). Conclusion: The adult KD is a rare disease that can have bad prognosis in short or long term and leave irreversible damage. The high rate of cardiac complication could be due to the long diagnosis delay, the absence of the gold standard treatment in 30% of cases or a selectional bias due to the difficulty to diagnose this disease in adulthood.
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Key words
Kawasaki disease,Aneurysms,Emergency care
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