Introduction: Kawasaki disease (KD) is a pan-vasculitis that primarily affects children and rarely adults. Available data on adult-onset KD are based on case reports and case series, both of which are limited by small sizes. We aimed to characterize the epidemiology, presentation, hospital course, and outcomes of adult-onset KD published in the English literature from 1980 through 2023. Methods: We retrospectively reviewed and included published articles with a diagnosis of KD in patients age ≥18 years. We searched PubMed and Google scholar for case reports/series published in English using the keywords “adult”, “Kawasaki disease”, and “mucocutaneous lymph node syndrome”. We extracted data from individual articles onto an Excel spreadsheet for analysis. Outcomes of interest were the demographic characteristics of patients, clinical presentation and hospital course, management, and complications. Descriptive statistics were used to analyze the data. Results: A total of 137 patients from 29 countries were included in this study. The majority were male (90/137, 64.9%; M:F ratio 1.8) and the median age was 25 years (range, 18-68). The median time to clinical presentation was 5 days (range, 4-60). The main signs and symptoms were fever (95.7%), skin rash (92.9%), conjunctivitis (89.3%), extremity changes (84.3%), oral changes (85.7%), and cervical lymphadenopathy (73.7%). The majority of the patients had no co-morbidities (125/137, 91.2%). EKG changes were not reported in 48 patients (35%). In the remaining 89, 26 (29.2%) had normal EKG or sinus tachycardia, 9 (10.1%) had ST segment elevation, and 9 (10.1%) had arrythmias or abnormal EKGs. Pharmacotherapy included IVIG (80/137, 58.4%), Aspirin (105/137, 76.6%), steroids (31/137, 22.6%) and antibiotics (68/137, 49.6%). For complications, 18.2% (25/137) had aneurysms, 8% (11/137) had myocardial infarction, 10.9% (15/137) had myocarditis, 10% had pericarditis/pericardial effusion, and 10.9% (15/137) had KD shock syndrome. The median length of stay was 14 days (range, 1-60 days). Three patients (2%) died from KD. Conclusion: Adult-onset KD is rare and is associated with significant cardiovascular complications. Clinicians should have high index of suspicion for prompt diagnoses and initiate appropriate treatment to improve outcomes.
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