Periodic fever syndromes: a patient diagnosed with recurrent Kawasaki disease

Abstract

<jats:title>Abstract</jats:title><jats:p>Kawasaki disease, known as mucocutaneous lymph node syndrome, is a multi-system disease of unknown aetiology that occurs in young children under 5 years of age. The recurrence rate of Kawasaki disease is as rare as 1–3%. Especially in cases with coronary artery involvement, recurrent Kawasaki disease should be investigated in terms of underlying rheumatologic diseases such as periodic fever syndromes, microscopic polyangiitis, polyarteritis nodosa, and systemic-onset juvenile arthritis. In this study, we report homozygote mutations in mevalonate kinase and familial Mediterranean fever genes in a recurrent Kawasaki disease with coronary dilatation.</jats:p>

Advances in understanding Kawasaki disease‐related immuno‐inflammatory response and vascular endothelial dysfunction

“…The rate of KD recurrence is 1%–3%; recurrence is most common within 12 months after the initial onset of the disease. 9 Moreover, patients with recurrent disease have an increased risk of coronary artery abnormalities, suggesting that innate immune memory enhances the immune response to heterologous reinfection signals or endogenous risk signals. 3 Immuno‐inflammatory mechanism in KD is shown in Figure 1 .…”

“…In patients with KD, the innate immune response is activated and the acquired immune system exhibits abnormal function. The rate of KD recurrence is 1%–3%; recurrence is most common within 12 months after the initial onset of the disease 9 . Moreover, patients with recurrent disease have an increased risk of coronary artery abnormalities, suggesting that innate immune memory enhances the immune response to heterologous reinfection signals or endogenous risk signals 3 .…”