Sugiura Yuki, Okumura Takahiro, Kondo Toru, Murohara Toyoaki
Department of Cardiology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8560, Japan.
Eur Heart J Case Rep. 2020 Sep 9;4(5):1-7. doi: 10.1093/ehjcr/ytaa304. eCollection 2020 Oct.
Kawasaki disease (KD) is a self-limiting form of systemic vasculitis. KD usually occurs in infants and young children and is rarely seen in adolescents. On rare occasions, KD is accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). The multifactorial causes of KDSS may include intensive vasculitis with capillary leak, myocardial dysfunction, and release of proinflammatory cytokines. However, the mechanisms underlying the pathophysiology of KDSS have not been fully elucidated.
A febrile 17-year-old male with cervical lymphadenopathy developed extreme shock with rapid cardiac dysfunction and reduced organ perfusion. Electrocardiogram revealed ST elevation in the precordial leads and increased serum levels of cardiac enzyme levels. Endomyocardial biopsy at the acute phase revealed CD3, CD4 or CD8, and CD20 lymphocytes and CD68 macrophages within infiltrates in the myocardium with mild interstitial fibrosis. He was treated with intravenous immunoglobulin (IVIG) and followed by glucocorticoids with mechanical circulatory support. His cardiac function recovered rapidly with no apparent adverse effects.
Our results suggest that KDSS may be a form of myocarditis, a condition in which inflammatory cells infiltrate the myocardium. Early immunosuppressive therapy, including IVIG and glucocorticoid therapy, may limit the severity of disease and improve the prognosis. As shown by this case, an accurate diagnosis of KD and KDSS will lead to early intervention and improved prognosis even among those in an older cohort.
川崎病（KD）是一种自限性的全身性血管炎。KD通常发生于婴幼儿，在青少年中罕见。极少数情况下，KD会因收缩期低血压导致器官灌注减少，这种情况称为川崎病休克综合征（KDSS）。KDSS的多因素病因可能包括伴有毛细血管渗漏的严重血管炎、心肌功能障碍以及促炎细胞因子的释放。然而，KDSS病理生理学的潜在机制尚未完全阐明。
一名17岁发热男性，伴有颈部淋巴结病，出现严重休克，伴有快速的心脏功能障碍和器官灌注减少。心电图显示胸前导联ST段抬高，血清心肌酶水平升高。急性期心内膜活检显示心肌浸润内有CD3、CD4或CD8淋巴细胞以及CD20淋巴细胞和CD68巨噬细胞，伴有轻度间质纤维化。他接受了静脉注射免疫球蛋白（IVIG）治疗，随后使用糖皮质激素并给予机械循环支持。他的心脏功能迅速恢复，无明显不良反应。
我们的结果表明，KDSS可能是心肌炎的一种形式，即炎症细胞浸润心肌的疾病。早期免疫抑制治疗，包括IVIG和糖皮质激素治疗，可能会限制疾病的严重程度并改善预后。如本病例所示，即使在年龄较大的队列中，准确诊断KD和KDSS也将导致早期干预并改善预后。
