Department of Pediatric Intensive Care Unit, Northwest Women's and Children's Hospital, Xi 'an, Shaanxi Province, China.
Medicine (Baltimore). 2024 Aug 2;103(31):e39199. doi: 10.1097/MD.0000000000039199.
Kawasaki disease (KD) is a vasculitis syndrome of small to medium-sized arteries that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. On rare occasions, it may accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). KDSS is a serious complication that can be presented to the emergency department as an initial feature when typical clinical symptoms of KD have not be detected.
We report the case of a 12-year-old boy admitted with prolonged fever, bilateral non-purulent conjunctivitis, and signs of shock such as hypotension and tachycardia. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and sterile pyuria. He was initially treated with intravenous cefotaxime and vancomycin considering the possible diagnosis of toxic shock syndrome, while the treatment was not effective. Subsequent chest computerized tomography and ultrasound identified pulmonary consolidation and polyserous effusion. Echocardiography revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function.
After a multidisciplinary consultation, a diagnosis of KDSS was made.
To prevent coronary artery lesions and other severe complications, the patient immediately received immunoglobulin, corticoid, and acetylsalicylic acid.
Soon afterwards, he showed significant improvement, with the temperature dropped to normal and hypotension corrected about 24 hours post-intravenous immunoglobulin therapy. Polyserous effusions also disappeared before discharge. Follow-up echocardiography revealed normal results.
Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. For children with KD, any symptoms pointing to infection should be carefully considered. When there is no etiologic evidence, antibiotics should be used with caution. Our case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition, timely treatment, and close monitoring are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS.
川崎病（KD）是一种中小动脉的血管炎综合征，具有典型的临床特征，如发热、皮疹、颈淋巴结肿大、结膜炎和黏膜改变。心脏表现，包括冠状动脉瘤、心肌炎、心肌梗死和心源性猝死，是 KD 观察到的最严重的并发症。在极少数情况下，它可能因收缩压低而伴有器官灌注减少，这种情况称为川崎病休克综合征（KDSS）。KDSS 是一种严重的并发症，当典型的 KD 临床症状尚未出现时，它可能会作为初始特征出现在急诊科。
我们报告了一例 12 岁男孩的病例，他因持续发热、双侧非脓性结膜炎和低血压、心动过速等休克体征入院。实验室检查发现炎症标志物升高、低白蛋白血症和无菌性脓尿。考虑到可能的中毒性休克综合征的诊断，他最初接受了静脉头孢噻肟和万古霉素治疗，但治疗无效。随后的胸部计算机断层扫描和超声检查发现肺实变和多浆膜腔积液。超声心动图显示轻度双心房扩张和轻度瓣膜反流，左心室功能正常。
经过多学科会诊，诊断为 KDSS。
为预防冠状动脉病变和其他严重并发症，患者立即接受免疫球蛋白、皮质类固醇和乙酰水杨酸治疗。
随后，他的病情迅速改善，体温降至正常，静脉注射免疫球蛋白治疗后 24 小时内低血压得到纠正。多浆膜腔积液也在出院前消失。随访超声心动图显示结果正常。
临床医生应保持对 KD 的高度警惕，并将肺部受累和多浆膜腔积液视为潜在并发症。对于 KD 患儿，任何提示感染的症状都应仔细考虑。当没有病因证据时，应谨慎使用抗生素。我们的病例还强调了在出现持续性发热和休克的儿童中，将 KDSS 作为鉴别诊断的重要性。早期识别、及时治疗和密切监测是预防严重并发症和确保 KDSS 患者良好结局的关键。
