Circulation. 2017 Apr 25;135(17):e927-e999. doi: 10.1161/CIR.0000000000000484. Epub 2017 Mar 29.
Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries.
To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.
These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances.
川崎病是一种儿童急性血管炎，如果未经治疗，约 25%的病例会导致冠状动脉瘤。该病已在全球范围内报道，是发达国家儿童获得性心脏病的主要病因。
为修订之前的美国心脏协会指南，召集了一个多学科专家写作小组，以审查和评估现有证据和基于实践的观点，并提供更新的诊断、急性疾病治疗和长期管理建议。尽管病因仍不清楚，但讨论部分强调了对该病流行病学、遗传学、发病机制、病理学、自然史和长期结局的新见解。及时诊断至关重要，更新的算法定义了辅助不完整经典临床标准诊断时使用的补充信息。虽然静脉注射免疫球蛋白是初始治疗的主要方法，但在某些患者中讨论了额外初始治疗的作用。约 10%至 20%的患者对初始静脉注射免疫球蛋白无反应，提供了额外治疗的建议。仔细管理不断演变的冠状动脉异常至关重要，需要增加评估频率和强化血栓预防。长期管理的风险分层主要基于最大冠状动脉内腔尺寸，归一化为 z 评分，并根据过去和当前的病变进行校准。有动脉瘤的患者需要终身不间断的心脏病学随访。
这些建议为诊断和管理川崎病的医疗保健提供者提供了更新的最佳循证指导，但临床决策应根据具体患者情况个体化。
