Objective

Adult-onset Still's disease (AOSD) is a rare inflammatory disease that may be life-threatening if complicated by cardiac problems. We performed a retrospective multicenter study to describe the manifestations, treatments and outcomes of cardiac involvement in AOSD.

Methods

We reviewed the medical databases of eight centers. All AOSD patients identified as fulfilling Yamagushi's or Fautrel's criteria were included in the study. Cardiac involvement, clinical manifestations, laboratory features, the course of the disease and treatments were evaluated.

Results

We included 96 AOSD patients in this study: 28 (29%) had documented cardiac involvement (AOSD + C group) and 68 (71%) had no cardiac involvement (control group). Cardiac complications were observed at diagnosis in 89% of cases. It were pericarditis (n = 17), tamponade (n = 5), myocarditis (n = 5) and non-infectious endocarditis (n = 1). Levels of leukocytes, neutrophils and C-reactive protein were significantly higher (p = 0.02, p = 0.02 and p = 0.002, respectively in the AOSD + C group than in the control group. Admission to intensive care, and the use of biotherapy were more frequent during follow-up in the AOSD + C group than the control group (p = 0.0001 and p = 0.03 respectively). Cardiac involvement was associated with refractory form in multivariate analyzed (p = 0.01). Corticosteroids were effective with or without methotrexate in 71% of patients but not in severe involvement as myocarditis or tamponade.

Conclusion

Cardiac complications are frequent, inaugural, can be life-threatening and predictive of a refractory course in patients with AOSD. Systematic cardiac screening should be proposed at diagnosis and biotherapy early use should be considered especially in myocarditis.

中文翻译：

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成人斯蒂尔病的心脏受累：一项对 28 名患者的回顾性研究的表现、治疗和结果。

客观的

成人斯蒂尔病 (AOSD) 是一种罕见的炎症性疾病，如果并发心脏问题，可能会危及生命。我们进行了一项回顾性多中心研究，以描述 AOSD 心脏受累的表现、治疗和结果。

方法

我们查阅了八个中心的医学数据库。所有被确定为符合 Yamagushi 或 Fautrel 标准的 AOSD 患者都包括在研究中。评估心脏受累、临床表现、实验室特征、病程和治疗。

结果

我们在本研究中纳入了 96 名 AOSD 患者：28 名 (29%) 有记录的心脏受累（AOSD + C 组），68 名（71%）没有心脏受累（对照组）。89% 的病例在诊断时观察到心脏并发症。分别为心包炎 ( n  = 17)、心包填塞 ( n  = 5)、心肌炎 ( n  = 5) 和非感染性心内膜炎 ( n  = 1)。 AOSD+C 组的白细胞、中性粒细胞和 C 反应蛋白水平显着高于对照组（分别为p  = 0.02、p  = 0.02 和p = 0.002）。 AOSD + C 组在随访期间比对照组更频繁（p  = 0.0001 和p  = 0.03）。在多变量分析中，心脏受累与难治性形式相关（p = 0.01）。在 71% 的患者中，无论是否使用甲氨蝶呤，皮质类固醇均有效，但在严重受累如心肌炎或心包填塞时无效。

结论

心脏并发症是常见的、首发的，可能危及生命，并且预示着 AOSD 患者的难治性病程。应在诊断时提出系统心脏筛查，并应考虑尽早使用生物治疗，尤其是在心肌炎中。