Goel Anurag Ratan, Yalcindag Ali
Department of Internal Medicine, The Warren Alpert Medical School of Brown University, Providence, RI, USA.
Department of Pediatrics, The Warren Alpert Medical School of Brown University, Providence, RI, USA.
Curr Rheumatol Rep. 2024 Dec 3;27(1):4. doi: 10.1007/s11926-024-01167-4.
To summarize advances in research on the epidemiology, pathogenesis, diagnosis, and treatment of Kawasaki Disease (KD), a systemic inflammatory illness of unknown etiology that affects children globally.
The epidemiology of KD was affected by the COVID-19 pandemic and advances in molecular immunology and machine learning have enabled research into its pathogenesis. There is ongoing research into agents that can be used to intensify initial treatment and accumulating evidence supporting the use of certain rescue regimens for refractory disease over others. There is promise surrounding a new coronary artery aneurysm prediction model. Research into the post-acute morbidity of KD continues. The COVID-19 pandemic temporarily reduced the incidence of KD. The NLRP3 inflammasome plays a key role in KD pathogenesis. Intensified initial treatment benefits high-risk patients, yet no intensification regimen shows superiority over another. Corticosteroids, infliximab, or combination therapy with IVIg plus another agent may be superior rescue regimens compared to IVIg alone for refractory KD. The Son score, developed in North America, predicted coronary artery lesions in Japanese and Italian cohorts. Patients with a history of KD may carry long-term physical and emotional burdens that persist into adulthood yet appear to have typical neurocognitive development. Successful transition to adult healthcare presents a challenge.
总结川崎病（KD）流行病学、发病机制、诊断及治疗方面的研究进展。KD是一种病因不明的全身性炎症性疾病，全球儿童均可受累。
KD的流行病学受到新冠疫情的影响，分子免疫学和机器学习的进展推动了其发病机制的研究。目前正在研究可用于强化初始治疗的药物，并且越来越多的证据支持某些难治性疾病抢救方案优于其他方案。一种新的冠状动脉瘤预测模型前景广阔。对KD急性后期发病率的研究仍在继续。新冠疫情使KD发病率暂时降低。NLRP3炎性小体在KD发病机制中起关键作用。强化初始治疗对高危患者有益，但尚无一种强化方案显示出比另一种更具优势。对于难治性KD，与单独使用静脉注射免疫球蛋白（IVIg）相比，皮质类固醇、英夫利昔单抗或IVIg加另一种药物的联合治疗可能是更优的抢救方案。在北美开发的Son评分可预测日本和意大利队列中的冠状动脉病变。有KD病史的患者可能会承受长期的身心负担，这些负担会持续到成年期，但似乎具有典型的神经认知发育。成功过渡到成人医疗保健是一项挑战。
