Gomard-Mennesson Emeline, Landron Cédric, Dauphin Claire, Epaulard Olivier, Petit Clemence, Green Lisa, Roblot Pascal, Lusson Jean-René, Broussolle Christiane, Sève Pascal
From Department of Internal Medicine (EGM, CP, LG, CB, PS) Hôtel-Dieu, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon; Department of Internal Medicine (CL, PR), Hôpital Jean Bernard, Poitiers; Department of Cardiology (CD, JRL), Hôpital Gabriel-Montpied, CHU Clermont-Ferrand; and Department of Infectious Diseases (OE), University Hospital, Grenoble, France.
Medicine (Baltimore). 2010 May;89(3):149-158. doi: 10.1097/MD.0b013e3181df193c.
Kawasaki disease (KD) is an acute multisystemic vasculitis occurring predominantly in children and rarely in adults. Diagnosis is made clinically using diagnostic guidelines; no specific test is available. "Incomplete" KD is a more recent concept, which refers to patients with fever lasting > or =5 days and 2 or 3 clinical criteria (rash, conjunctivitis, oral mucosal changes, changes of extremities, adenopathy), without reasonable explanation for the illness. To describe the clinical and laboratory features of classical (or "complete") KD, and incomplete KD in adults, we report 10 cases of adult KD, including 6 patients who fulfilled the criteria for incomplete KD, diagnosed either at presentation (n = 4) or retrospectively (n = 2). At the time of clinical presentation, complete KD was diagnosed in 4 patients, while 4 patients fulfilled the criteria for incomplete KD. For 3 of the 4 patients with incomplete KD, presence of severe inflammation, laboratory findings (hypoalbuminemia, anemia, elevation of alanine aminotransferase, thrombocytosis after 7 days, white blood cell count > or =15,000/mm, and urine > or =10 white blood cell/high power field), or echocardiogram findings were consistent with the diagnosis. In 2 patients, the diagnosis of KD was made retrospectively in the presence of myocardial infarction due to coronary aneurysms, after an undiagnosed medical history evocative of incomplete KD. Seven patients received intravenous immunoglobulins (IVIG), after a mean delay of 12.5 days, which appeared to shorten the course of the disease. This relatively large series of adult KD highlights the existence of incomplete KD in adults and suggests that the algorithm proposed by a multidisciplinary committee of experts to diagnose incomplete KD in children could be useful in adults. Further studies are needed to determinate whether prompt IVIG may avoid artery sequelae in adult patients with complete or incomplete KD.
川崎病(KD)是一种主要发生于儿童、极少见于成人的急性多系统血管炎。临床诊断依据诊断指南进行;尚无特异性检测方法。“不完全性”KD是一个较新的概念,指发热持续≥5天且有2或3项临床标准(皮疹、结膜炎、口腔黏膜改变、四肢改变、淋巴结病)而病因不明的患者。为描述成人经典型(或“完全性”)KD及不完全性KD的临床和实验室特征,我们报告10例成人KD病例,其中6例符合不完全性KD标准,4例初诊时确诊,2例为回顾性诊断。临床表现时,4例诊断为完全性KD,4例符合不完全性KD标准。4例不完全性KD患者中,3例存在严重炎症、实验室检查结果(低白蛋白血症、贫血、丙氨酸转氨酶升高、7天后血小板增多、白细胞计数≥15,000/mm³及尿白细胞≥10个/高倍视野)或超声心动图表现符合诊断。2例患者在有未确诊的不完全性KD病史且出现冠状动脉瘤导致的心肌梗死后回顾性诊断为KD。7例患者平均延迟12.5天后接受静脉注射免疫球蛋白(IVIG)治疗,似乎缩短了病程。这一相对较大系列的成人KD病例突出了成人不完全性KD的存在,并表明多学科专家委员会提出的儿童不完全性KD诊断算法对成人可能有用。需要进一步研究以确定及时给予IVIG是否可避免成人完全性或不完全性KD患者出现动脉后遗症。