Sánchez-Manubens J, Gelman A, Franch N, Teodoro S, Palacios J R, Rudi N, Rivera J, Antón J
Pediatric Rheumatology Unit, Pediatrics Department, Hospital Parc Taulí Sabadell, Barcelona, Spain.
Pediatric Rheumatology Unit, Pediatrics Department, Hospital Sant Joan de Déu Esplugues, Barcelona, Spain.
BMC Pediatr. 2017 Apr 8;17(1):102. doi: 10.1186/s12887-017-0852-6.
Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology. Intravenous immunoglobulin (IVIG) is an effective treatment and decreases the risk of cardiac complications to less than 5%. In spite of its effectiveness, some children do not respond to this therapy and still develop coronary aneurysms (CAA). The optimal treatment for IVIG non-responsive patients remains controversial although corticoids have been suggested to be an effective treatment in some patients. For those patients still resistant to IVIG and corticoids, interleukin-1 receptor antagonists (IL-1RA) such anakinra could be an alternative.
We present a 3 year-old Caucasian patient with KD without cardiac complications but with important resistance to treatment. After becoming resistant to IVIG and corticoids, anakinra proved to be an effective treatment.
To our knowledge, this is the first report of the utility of IL-1RA in refractory KD without coronary impairment. The patient fulfilled the classical criteria for KD and, after becoming resistant to first and second line treatments, anakinra proved to be an effective treatment. Further studies are required to determine if this is an effective treatment option for other cases of resistant Kawasaki disease.
川崎病(KD)是一种病因不明的急性自限性全身性血管炎。静脉注射免疫球蛋白(IVIG)是一种有效的治疗方法,可将心脏并发症的风险降低至5%以下。尽管其疗效显著,但一些儿童对这种治疗无反应,仍会发生冠状动脉瘤(CAA)。尽管有人认为皮质类固醇对某些患者是一种有效的治疗方法,但IVIG无反应患者的最佳治疗方法仍存在争议。对于那些对IVIG和皮质类固醇仍有抵抗的患者,白细胞介素-1受体拮抗剂(IL-1RA)如阿那白滞素可能是一种替代方法。
我们报告一名3岁的白种人川崎病患者,无心脏并发症,但对治疗有重要抵抗。在对IVIG和皮质类固醇产生抵抗后,阿那白滞素被证明是一种有效的治疗方法。
据我们所知,这是IL-1RA在无冠状动脉损伤的难治性川崎病中应用的首例报告。该患者符合川崎病的经典标准,并在对一线和二线治疗产生抵抗后,阿那白滞素被证明是一种有效的治疗方法。需要进一步研究以确定这是否是其他难治性川崎病病例的有效治疗选择。