A Systematic Review of Atypical Presentations of Kawasaki Disease

Background: Kawasaki disease (KD) is a systematic vasculitis that mainly affects children under five years old. Diagnosis is made based on clinical criteria. Objectives: This study aimed to determine various atypical presentations of KD to prevent misdiagnosis. Methods: A comprehensive systematic search in PubMed, Web of Science, and Scopus databases was performed based on the PRISMA guidelines from 2012 to January 2023. Case reports and case series studies reporting the atypical presentations of KD, only in the English language, were included, and the Joanna Briggs Institute (JBI) checklist was utilized for case report studies as a quality assessment tool. Results: Our findings revealed that patients with KD can present with gastrointestinal and hepatobiliary, cardiovascular, mucocutaneous, neurological, musculoskeletal, respiratory, retropharyngeal, and renal complications. Macrophage activation syndrome and normal inflammatory biomarkers were rare manifestations. The most common cause of the patient’s death was cardiovascular manifestations, especially myocardial infarction. Cardiovascular and gastrointestinal involvement were the most prevalent. Conclusion: Focusing on atypical and rare manifestations facilitates the correct diagnosis, leading to timely and appropriate management, improving outcomes, and reducing complications.