Kawasaki Disease Vasculitis: From Diagnosis to New Concepts in Pathophysiology and Therapeutic Approaches

Kawasaki disease (KD) is an acute, self-limiting vasculitis that primarily affects children under 5 years old. KD manifests as a persistent fever in the presence of mucocutaneous inflammation and lymphadenopathy, which in severe cases leads to the development of coronary artery aneurysms (CAAs). While early intervention with high-dose intravenous immunoglobulin and aspirin significantly lowers the risk of CAAs, up to 20% of patients with KD are resistant to intravenous immunoglobulin and face a substantially higher risk of developing coronary complications, highlighting the urgent need for more effective adjunctive and rescue therapies. Moreover, coronary abnormalities may persist after the apparent resolution of aneurysms, and cardiac complications extend into adolescence and adulthood. Murine models mimicking KD vasculitis have played a pivotal role in advancing our understanding of the disease's immunopathology, shedding light on the immune mechanisms driving its cardiovascular complications. Here, we summarize the current understanding of KD immunopathogenesis and its cardiovascular complications, as well as recent preclinical findings that are facilitating the development of novel therapeutic strategies, offering hope for improved management of KD in the future.