Heart Center, Children's Hospital of Fudan University, National Children's Medical Center, 399 Wan Yuan Road, Shanghai, 201102, China.
Pediatr Rheumatol Online J. 2021 Nov 8;19(1):157. doi: 10.1186/s12969-021-00643-w.
Kawasaki disease (KD) is a systemic vasculitis that predominantly affects medium-sized arteries. In addition to well-known coronary artery aneurysms (CAAs), peripheral systemic artery aneurysms (SAAs) have also been sporadically reported. In the literatures, SAAs occurred mainly in untreated, intravenous immunoglobin (IVIG)-resistant, or severe refractory KD, and thrombotic events in SAAs were rarely reported.
A 10-month-old boy with a history of KD was referred to our hospital for suspected pseudoaneurysm of the axillary arteries. Four months prior to presentation, he had persistent fever, conjunctival congestion, and rash. On the 10th day of fever echocardiogram showed biliteral CAAs. He was then diagnosed with KD and given IVIG 2 g/kg and aspirin at a local hospital. His fever and symptoms soon subsided and he was discharged with low dose aspirin and dipyridamole. One month prior to presentation, his parents incidentally palpated swellings in his bilateral axillae. On admission, physical examination revealed a pulsatile swelling in his right axilla and a non-pulsatile swelling in the left with impalpable left brachial and radial pulses, cooler and less active left upper limb than the right one. While the pulses of other three limbs were normal. Ultrasound examination revealed giant bilateral axillary artery aneurysms (AAAs) with massive thrombus in the left. Angiography confirmed giant bilateral AAAs with left AAAs completely occluded and fine collateral vessels connecting to the distal brachial artery, in addition to giant bilateral multiple CAAs without stenoses. The patient was given intravenous prostaglandin for 10 days to allow for formation of collateral circulation, as well as aspirin, low molecular weight heparin (which was switched to warfarin before discharge) and metoprolol. At discharge, the temperature and movement of his left upper limb improved significantly. On follow-up at 7 months, his left upper limb further improved and was similar to the right with no occurrence of cardiovascular events. The images of CAAs and AAAs on echocardiogram and computerized tomography remained the same.
This case highlights the importance of evaluating peripheral SAAs in KD patients with CAAs, even if their course of treatment appears smooth. For both large non-aortic SAAs and CAAs in KD patients, antithrombotic therapy is of utmost importance.
川崎病（KD）是一种主要影响中型动脉的全身性血管炎。除了众所周知的冠状动脉瘤（CAA）外，外周系统性动脉瘤（SAA）也时有报道。文献中，SAA 主要发生在未经治疗、静脉免疫球蛋白（IVIG）耐药或严重难治性 KD 患者中，SAA 中的血栓形成事件很少报道。
一名 10 个月大的男孩，有 KD 病史，因疑似腋动脉假性动脉瘤而被转至我院。在就诊前 4 个月，他持续发热、结膜充血和皮疹。发热第 10 天的超声心动图显示双侧 CAA。他随后被诊断为 KD，并在当地医院接受了 2g/kg 的 IVIG 和阿司匹林治疗。他的发热和症状很快消退，出院时服用小剂量阿司匹林和双嘧达莫。在就诊前 1 个月，他的父母偶然发现他双侧腋窝有肿胀。入院时，体格检查发现他的右侧腋部有搏动性肿胀，左侧有非搏动性肿胀，左侧肱动脉和桡动脉脉搏难以触及，左侧上肢较右侧上肢发凉、活动减少。其他四肢的脉搏正常。超声检查显示双侧巨大腋动脉动脉瘤（AAA），左侧有大量血栓。血管造影证实双侧巨大 AAA，左侧 AAA 完全闭塞，有细小的侧支血管与远端肱动脉相连，此外还有双侧多发性巨大 CAA，无狭窄。给予患者静脉前列腺素治疗 10 天，以形成侧支循环，同时给予阿司匹林、低分子肝素（出院前换用华法林）和酒石酸美托洛尔。出院时，他的左上肢体温和活动度明显改善。7 个月后随访时，他的左上肢进一步改善，与右侧相似，无心血管事件发生。超声心动图和计算机断层扫描上的 CAA 和 AAA 图像保持不变。
本例强调了在有 CAA 的 KD 患者中评估外周 SAA 的重要性，即使他们的治疗过程看起来顺利。对于大型非主动脉 SAA 和 KD 患者的 CAA，抗血栓治疗至关重要。
