Lucron H, Bosser G, Lethor J P, Sommelet D, Feillet F, Burger G, Monin P, Marçon F
Service de cardiologie pédiatrique, hôpital d'enfants, CHU de Brabois, Vandoeuvre-lès-Nancy.
Arch Mal Coeur Vaiss. 2004 May;97(5):522-8.
We studied 52 consecutive patients with Kawasaki disease hospitalized (1984 -2003) during the acute phase (mean age 2.5 + 2.4 years; range 0.3 to 16 years, 34 males, 18 cases with coronary aneurysms, median follow-up 6.7 years), and identified a subgroup presenting a refractory subtype to immunoglobulin therapy.
forty-nine infants benefited from a first regimen of immunoglobulins, 8.4 + 6 days following the onset of symptoms. Eleven infants (1.4 + 1.2 years, range 0.3 - 4.3 years, median 1.7 years) were non-responders, with coronary aneurysms in 8 cases (giant aneurysms (>8 mm) in 4 cases). These 11 infants were treated a second time by immunoglobulins, but 6 cases (1.8 + 1.6 years, with two cases of severe ventricular dysfunction and 2 cases of fatal myocardial infarction) required an additive therapy with (oral or IV route) corticosteroids (2) and cyclophosphamide bolus (4) with or without repetitive plasmapheresis (4). Non-responder patients had their treatment onset later (p<0.0003) using higher dosages (p<0.005), a longer delay for fever or biological signs correction (p<0.02), a worsening of coronary lesions (p<0.05) with more coronary secondary aneurysms (p<.005). The aneurysms, more frequent at the second phase of the disease (p<0.0001) are associated with: a younger age (p<0.03), a lower weight (p<0.02), a later onset of treatment (p<0.03), prolonged fever or inflammatory syndrome (p<0.05), higher level of fibrinogene (p<0.02). The overall mortality (5.7%) is correlated with giant aneurysms (p<0.001), myocardial ischemia (p<0.0001), heart failure (p<0.0001), and lack of early response to treatment (p<0.003).
immunoglobin therapy can be repeated. In case of severe forms, the use of corticosteroids, cyclophosphamide and plasmapheresis may be proposed.
我们研究了1984年至2003年期间急性期住院的52例川崎病连续患者（平均年龄2.5±2.4岁；范围0.3至16岁，男性34例，18例有冠状动脉瘤，中位随访6.7年），并确定了一个对免疫球蛋白治疗呈难治性亚型的亚组。
49例婴儿受益于首次免疫球蛋白治疗方案，症状出现后8.4±6天开始治疗。11例婴儿（1.4±1.2岁，范围0.3至4.3岁，中位年龄1.7岁）无反应，其中8例有冠状动脉瘤（4例为巨大动脉瘤（>8mm））。这11例婴儿第二次接受免疫球蛋白治疗，但6例（1.8±1.6岁，2例严重心室功能障碍，2例致命性心肌梗死）需要联合（口服或静脉途径）皮质类固醇（2例）和环磷酰胺大剂量冲击（4例）治疗，伴或不伴重复血浆置换（4例）。无反应患者治疗开始时间较晚（p<0.0003），使用剂量较高（p<0.005），发热或生物学指标纠正延迟较长（p<0.02），冠状动脉病变恶化（p<0.05），冠状动脉继发性动脉瘤更多（p<0.005）。动脉瘤在疾病的第二阶段更常见（p<0.0001），与以下因素有关：年龄较小（p<0.03）、体重较低（p<0.02）、治疗开始较晚（p<0.03）、发热或炎症综合征持续时间较长（p<0.05）、纤维蛋白原水平较高（p<0.02）。总体死亡率（5.7%）与巨大动脉瘤（p<0.001）、心肌缺血（p<0.0001）、心力衰竭（p<0.0001）以及对治疗缺乏早期反应（p<0.003）相关。
免疫球蛋白治疗可以重复。对于严重病例，可考虑使用皮质类固醇、环磷酰胺和血浆置换。
