Mastrolia Maria Vincenza, Abbati Giulia, Signorino Claudia, Maccora Ilaria, Marrani Edoardo, Pagnini Ilaria, Simonini Gabriele
Rheumatology Unit, Meyer Children's University Hospital, Firenze, Italy.
Pediatric Residency, Meyer Children's University Hospital, University of Florence, Firenze, Italy.
Ther Adv Musculoskelet Dis. 2021 Mar 29;13:1759720X211002593. doi: 10.1177/1759720X211002593. eCollection 2021.
Refractory Kawasaki disease (KD) is related to a major risk of coronary arteries abnormalities and its treatment is not standardized. In this regard, anakinra (ANA), an interleukin (IL)-1 receptor antagonist, represents an emerging therapeutic option. We report two cases of children, diagnosed with KD, nonresponsive to two doses of intravenous immunoglobulins, successfully treated with ANA, without a prior use of steroids. Patient 2 developed a coronary dilatation, that improved significantly after ANA therapy. Our experience highlights IL-1 blockade effectiveness in reducing KD inflammation and suggests ANA adoption as second-line therapy, with a timesaving and steroid-sparing strategy. Our results, combined with the evidence of the IL-1 key role in KD and coronary arteritis pathogenesis and to the recent clinical evidence reported by the KAWAKINRA trial, encourage an earlier recourse to ANA in patients with refractory KD, in order to fight inflammation, and to treat and prevent the development of coronary artery aneurysms. Further studies are needed to better define the place of IL-1 blockade in KD step-up treatment.
难治性川崎病（KD）与冠状动脉异常的主要风险相关，其治疗并不规范。在这方面，阿那白滞素（ANA），一种白细胞介素（IL）-1受体拮抗剂，代表了一种新兴的治疗选择。我们报告了两例被诊断为KD的儿童病例，这两名儿童对两剂静脉注射免疫球蛋白均无反应，但在未预先使用类固醇的情况下，使用ANA成功治愈。病例2出现了冠状动脉扩张，在ANA治疗后显著改善。我们的经验突出了IL-1阻断在减轻KD炎症方面的有效性，并建议采用ANA作为二线治疗方法，以节省时间并避免使用类固醇。我们的结果，结合IL-1在KD和冠状动脉炎发病机制中的关键作用的证据以及KAWAKINRA试验报告的最新临床证据，鼓励对难治性KD患者更早地使用ANA，以对抗炎症，并治疗和预防冠状动脉瘤的发展。需要进一步的研究来更好地确定IL-1阻断在KD强化治疗中的地位。
