Department of BioMedicine, Section of Rheumatology, Transition Clinic, University of Florence, Florence, Italy.
Pediatr Rheumatol Online J. 2011 Jul 20;9:17. doi: 10.1186/1546-0096-9-17.
More than 40 years have passed since Kawasaki syndrome (KS) was first described. Yet KS still remains an enigmatic illness which damages the coronary arteries in a quarter of untreated patients and is the most common cause of childhood-acquired heart disease in developed countries. Many gaps exist in our knowledge of the etiology and pathogenesis of KS, making improvements in therapy difficult. In addition, many KS features and issues still demand further efforts to achieve a much better understanding of the disease. Some of these problem areas include coronary artery injuries in children not fulfilling the classic diagnostic criteria, genetic predisposition to KS, unpredictable ineffectiveness of current therapy in some cases, vascular dysfunction in patients not showing echocardiographic evidence of coronary artery abnormalities in the acute phase of KS, and risk of potential premature atherosclerosis. Also, the lack of specific laboratory tests for early identification of the atypical and incomplete cases, especially in infants, is one of the main obstacles to beginning treatment early and thereby decreasing the incidence of cardiovascular involvement. Transthoracic echocardiography remains the gold-standard for evaluation of coronary arteries in the acute phase and follow-up. In KS patients with severe vascular complications, more costly and potentially invasive investigations such as coronary CT angiography and MRI may be necessary. As children with KS with or without heart involvement become adolescents and adults, the recognition and treatment of the potential long term sequelae become crucial, requiring that rheumatologists, infectious disease specialists, and cardiologists cooperate to develop specific guidelines for a proper evaluation and management of these patients. More education is needed for physicians and other professionals about how to recognize the long-term impact of systemic problems related to KS.
川崎病（KS）被首次描述已经过去 40 多年了。然而，KS 仍然是一种神秘的疾病，四分之一未经治疗的患者会损害冠状动脉，是发达国家儿童获得性心脏病的最常见原因。我们对 KS 的病因和发病机制的认识还存在许多空白，这使得治疗的改进变得困难。此外，KS 的许多特征和问题仍然需要进一步努力，以更好地了解这种疾病。其中一些问题领域包括不符合经典诊断标准的儿童的冠状动脉损伤、KS 的遗传易感性、某些情况下当前治疗方法的不可预测无效性、在 KS 急性期没有超声心动图冠状动脉异常证据的患者的血管功能障碍、以及潜在的动脉粥样硬化过早发生的风险。此外，缺乏用于早期识别非典型和不完整病例的特定实验室检测，尤其是在婴儿中，这是早期开始治疗从而降低心血管受累发生率的主要障碍之一。经胸超声心动图仍然是评估急性期和随访期冠状动脉的金标准。对于有严重血管并发症的 KS 患者，可能需要更昂贵和潜在侵入性的检查，如冠状动脉 CT 血管造影和 MRI。随着患有或不伴有心脏受累的 KS 儿童成长为青少年和成年人，识别和治疗潜在的长期后遗症变得至关重要，这需要风湿病学家、传染病专家和心脏病专家合作，为这些患者制定特定的评估和管理指南。需要对医生和其他专业人员进行更多关于如何识别与 KS 相关的系统性问题的长期影响的教育。
