Abstract：
Myocarditis has been reported as a life-threatening complication of adult-onset Still\'s disease (AOSD), but fulminant myocarditis with AOSD is very rare. We hereby report a case of a 43-year-old female with fulminant myocarditis with AOSD. She had a refractory AOSD and cardiogenic shock with markedly elevated ferritin level up to 67,370 ng/mL. She was successfully treated with canakinumab and mechanical circulatory support (MCS) such as venoarterial extracorporeal membrane oxygenation and Impella CP. We also reviewed the previous cases of fulminant myocarditis with AOSD published from 1976 to December 2022, and only 8 cases of fulminant myocarditis with AOSD have been reported. The characteristics of these cases showed that the average age at presentation was 37.6 years (range 24-47 years). The time to myocarditis from the onset of AOSD ranged from 2 weeks to 2 years; however, most cases developed myocarditis within 1 year. Initial presenting symptoms included fever, dyspnea, chest pain, myalgia, rash, and sore throat. The median peak ferritin was 13,000 ng/mL. Left ventricular ejection fractions were not greater than 35%. Our case was the first reported case successfully treated with canakinumab and MCS. This review suggests that myocarditis may be an early phase of the complication in patients with AOSD, and the severity of AOSD may correlate with the severity of myocarditis. Canakinumab for AOSD and MCS for fulminant myocarditis may be one of the choices for overcoming the comorbidities.
摘要：
心肌炎已被报道为成人发作的斯蒂尔病（AOSD）的危及生命的并发症，但是伴有AOSD的暴发性心肌炎非常罕见。我们在此报告一例43岁女性患有AOSD的暴发性心肌炎。她患有难治性AOSD和心源性休克，铁蛋白水平显着升高，高达67,370ng/mL。她成功接受了canakinumab和机械循环支持（MCS）治疗，例如静脉动脉体外膜氧合和ImpellaCP。我们还回顾了1976年至2022年12月发表的AOSD暴发性心肌炎病例，仅报道了8例AOSD暴发性心肌炎。这些病例的特征表明，就诊时的平均年龄为37.6岁（范围24-47岁）。从AOSD发作到心肌炎的时间从2周到2年不等；然而，大多数病例在1年内发展为心肌炎。最初出现的症状包括发烧，呼吸困难,胸痛,肌痛，皮疹,喉咙痛.铁蛋白的中值峰值为13,000ng/mL。左心室射血分数不大于35%。我们的病例是第一个成功使用canakinumab和MCS治疗的病例。本综述提示心肌炎可能是AOSD患者并发症的早期阶段。AOSD的严重程度可能与心肌炎的严重程度有关。用于AOSD的Canakinumab和用于暴发性心肌炎的MCS可能是克服合并症的选择之一。
