AOU Meyer and University of Florence, 50139 Florence, Italy.
Best Pract Res Clin Rheumatol. 2009 Oct;23(5):689-97. doi: 10.1016/j.berh.2009.08.010.
Kawasaki disease (KD) is the most common systemic vasculitis in childhood after Henoch-Schonlein purpura, and the most common cause of acquired heart disease among children living in Western countries. Its diagnosis relies on clinical findings; laboratory tests are useful to rule out other causes of unexplained fever but are not specific for the diagnosis of KD. Numerous efforts to produce a diagnostic algorithm have been made, but without success. Expert opinion is therefore required in doubtful cases, especially those that lack classical criteria (the so-called atypical or incomplete cases). Renal, gastrointestinal, neurologic, pulmonary and ocular involvements have all been described. Infants may be at higher risk of complications since recognising manifestations of the disease might be more difficult in this group. Approaches to treatment and follow-up of KD are changing in parallel with changes in concepts of what constitutes classical and incomplete KD. Guiding this evolution is the probability that the diagnosis is actually KD, the duration of the child's illness and the desired effects of therapy. Until a gold standard for diagnosing KD is available, these therapeutic decisions will continue to be made on an individual basis.
川崎病（KD）是继过敏性紫癜后儿童期第二大全身性血管炎，也是西方国家儿童后天性心脏病的主要病因。KD 的诊断依赖于临床发现；实验室检查有助于排除不明原因发热的其他病因，但对 KD 的诊断无特异性。人们已经做出了诸多尝试来制定诊断算法，但均未成功。因此，在可疑病例中需要专家意见，尤其是那些缺乏典型标准（即所谓的不典型或不完全病例）的病例。肾脏、胃肠道、神经、肺和眼部受累均有报道。由于在该组中可能更难识别疾病的表现，因此婴儿可能面临更高的并发症风险。KD 的治疗和随访方法正在随着对经典型和不典型 KD 的认识的变化而变化。指导这一演变的是实际诊断为 KD 的可能性、患儿疾病的持续时间和治疗的预期效果。在获得 KD 的金标准之前，这些治疗决策将继续根据个体情况做出。
