Immunoglobulin-resistant Kawasaki disease.

Bibiana Marriaga-Núñez, Araceli Arellano-Valdez, Juan P Abarca-de la Paz, Miguel A Bonal-Pérez, Jesús G Montaño-Durón, Fortino Solórzano-Santos
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Abstract

Background: Kawasaki disease is a systemic vasculitis that affects small and medium-sized vessels, primarily the coronary arteries. First-line treatment includes intravenous immunoglobulin (IVIG) and acetylsalicylic acid; however, 20% do not respond adequately despite treatment. We describe a case treated with etanercept after initial IVIG failure, showing a good response.

Case report: A 5-year-old female was diagnosed with classic Kawasaki disease. Echocardiography and angiotomography revealed giant and fusiform aneurysms in the coronary arteries. A first dose of IVIG therapy was administered without improvement; after the second dose, the fever persisted, so etanercept was administered, and the fever subsided. There were no new lesions in medium-caliber vessels and the previously identified coronary lesions did not progress.

Conclusions: The use of etanercept in Kawasaki disease has demonstrated a clinically favorable response. Controlled clinical trials of this drug are needed to establish it as a formal therapy in cases of initial IVIG failure.

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免疫球蛋白耐药性川崎病。
背景:川崎病是一种系统性血管炎,影响中小血管,主要是冠状动脉。一线治疗包括静脉注射免疫球蛋白(IVIG)和乙酰水杨酸;然而,20%的患者尽管接受了治疗,但仍没有充分的反应。我们描述了一例首次IVIG失败后用依那西普治疗的病例,显示出良好的反应。病例报告:一名5岁女性被诊断为典型川崎病。超声心动图和血管断层扫描显示冠状动脉中有巨大的梭形动脉瘤。第一剂IVIG治疗没有改善;第二次给药后,发烧持续,因此服用依那西普,发烧消退。中等口径血管中没有新的病变,先前确定的冠状动脉病变也没有进展。结论:依那西普治疗川崎病具有良好的临床疗效。需要对这种药物进行对照临床试验,以将其作为初始IVIG失败的正式治疗方法。
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来源期刊
CiteScore
1.60
自引率
0.00%
发文量
73
审稿时长
20 weeks
期刊介绍: The Boletín Médico del Hospital Infantil de México is a bimonthly publication edited by the Hospital Infantil de México Federico Gómez. It receives unpublished manuscripts, in English or Spanish, relating to paediatrics in the following areas: biomedicine, clinical, public health, clinical epidemology, health education and clinical ethics. Articles can be original research articles, in-depth or systematic reviews, clinical cases, clinical-pathological cases, articles about public health, letters to the editor or editorials (by invitation).
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