OBJECTIVE To evaluate the cardiac involvement in patients with Still's disease with a focus on myocarditis included in the multicenter AIDA (AutoInflammatory Disease Alliance) network Still's disease registry. To exploit the predictive factors for myocarditis in deriving a clinical risk patient profile for this severe manifestation. METHODS A multicenter observational study was built up assessing consecutive patients with Still's disease characterized by the cardiac involvement among those included in the AIDA Network Still's Disease Registry. The cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis. RESULTS In total, 73 patients with Still's disease and cardiac involvement were assessed (mean age 36.3±19.9 years, 42.5% male sex); out of them, 21.9% were children. The most common cardiac manifestation was the pericarditis in 90.4% of patients, 26.0% presented with myocarditis, and less frequently endocarditis (2.7%) and tamponade (1.4%). Comparing clinical features of patients with myocarditis than others, significantly increased frequencies of skin rash, and pleuritis as well as higher values of systemic score were recognised. Furthermore, an enhanced mortality rate was registered in patients with myocarditis. In regression models, the skin rash and the systemic score independently predicted the myocarditis. CONCLUSION The characteristics of patients with Still's disease and cardiac involvement were assessed in the AIDA network. The most common feature was the pericarditis but also a more severe clinical picture was reported in patients with myocarditis. The latter was associated with increased mortality rate and with higher systemic score, identifying patients to be carefully managed.

中文翻译：

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斯蒂尔病患者心肌炎的评估;来自多中心国际 AIDA 网络 Still's Disease Registry 的临床发现。


目的 评估 Still's 病患者的心脏受累情况，重点是 Still's 病 网络 Still's 病登记处中包括的心肌炎。利用心肌炎的预测因素得出这种严重表现的临床风险患者概况。方法 建立了一项多中心观察性研究，评估了 AIDA 网络 Still's 疾病登记处中以心脏受累为特征的连续 Still's 病患者。根据心包炎、心包填塞、心肌炎和/或无菌性心内膜炎的存在来定义心脏受累。结果 共评估了 73 例斯蒂尔病和心脏受累患者 （平均年龄 36.3±19.9 岁，42.5% 为男性）;其中，21.9% 是儿童。最常见的心脏表现是心包炎，90.4% 的患者，26.0% 的患者表现为心肌炎，较少见的是心内膜炎 （2.7%） 和填塞 （1.4%）。比较心肌炎患者的临床特征，发现皮疹和胸膜炎的发生率显著增加，全身评分值较高。此外，心肌炎患者的死亡率增加。在回归模型中，皮疹和全身评分独立预测心肌炎。结论 在 AIDA 网络中评估 Still's 病和心脏受累患者的特征。最常见的特征是心包炎，但在心肌炎患者中也报告了更严重的临床表现。后者与死亡率增加和全身评分升高相关，确定了需要仔细管理的患者。