Division of Immunology, Boston Children's Hospital, Boston, MA, USA.
Division of Immunology, Boston Children's Hospital, Boston, MA, USA; Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
Lancet Child Adolesc Health. 2024 Oct;8(10):781-792. doi: 10.1016/S2352-4642(24)00169-X.
Kawasaki disease is a paediatric vasculitis that presents with fever, rash, conjunctivitis, mucositis, lymphadenopathy, and extremity changes, and primarily affects children younger than 5 years. Coronary artery aneurysms are observed in approximately 20% of patients without treatment. Giant coronary artery aneurysms are rare but can result in substantial morbidity and mortality due to the risk of thrombosis, stenosis, and myocardial infarction. Infants younger than 6 months and children with coronary artery abnormalities are at highest risk for the development of large or giant coronary artery aneurysms, necessitating swift identification and aggressive treatment. The children at high risk for coronary artery aneurysms warrant primary intensification therapy; however, what the most optimal adjunct therapy might be to reduce their risk is unclear and large-scale international trials are needed. Kawasaki disease is a clinical diagnosis that shares many features with other common febrile illnesses, including multisystem inflammatory syndrome in children. Identifying biomarkers that can distinguish Kawasaki disease from similar conditions and predict coronary artery aneurysm risk are needed to aid timely diagnosis, guide management, and improve patient outcomes.
川崎病是一种儿科血管炎，表现为发热、皮疹、结膜炎、黏膜炎症、淋巴结病和四肢变化，主要影响 5 岁以下儿童。未经治疗的患者中约有 20%出现冠状动脉瘤。巨大冠状动脉瘤很少见，但由于血栓形成、狭窄和心肌梗死的风险，可能导致严重的发病率和死亡率。6 个月以下的婴儿和有冠状动脉异常的儿童发生大或巨大冠状动脉瘤的风险最高，需要迅速识别和积极治疗。有发生冠状动脉瘤风险的儿童需要进行主要强化治疗；然而，降低其风险的最佳辅助治疗方法尚不清楚，需要开展大规模的国际试验。川崎病的临床诊断与其他常见发热性疾病（包括儿童多系统炎症综合征）有许多共同特征。需要确定能够区分川崎病与类似疾病并预测冠状动脉瘤风险的生物标志物，以帮助及时诊断、指导治疗和改善患者预后。
