53例成人Still病临床特征分析及文献复习

Analysis of clinical characteristics of fifty-three patients with adult onset still disease and literature review

目的:通过对53例成人Still病例临床资料进行回顾性分析,以提高对该病的诊疗水平。方法:回顾性分析我院53例成人Still病患者的临床资料、辅助检查和疾病预后转归情况。结果:在回顾性分析中,女性患者较男性患者例数更多(52.8%vs.47.2%),发生严重并发症如多器官功能损害、噬血综合征等的患者预后较差。基线降钙素原、白介素-6、C反应蛋白、血沉、白细胞计数、中性粒细胞百分比在不同预后患者之间无明显差异。预后好患者治疗后铁蛋白水平明显低于预后差患者[(4 898±8 938)μg/L vs.(31 810±17 710)μg/L,P<0.001],预后好患者在治疗后铁蛋白水平较治疗前明显下降[(4 898±8 938)μg/L vs.(14 270±19 070)μg/L,P<0.001],预后差患者治疗后铁蛋白水平较治疗前仍持续上升[(31 810±17 710)μg/L vs.(15 240±22 150)μg/L,P<0.001]。结论:成人Still病是以发热、皮疹、关节疼痛为主要特征的一类疾病,铁蛋白对患者的诊断和治疗具有较好的预测效果,大部分患者通过治疗预后较好,少部分出现严重并发症的患者预后差。

Objective：To improve the diagnosis and treatment of adult onset still disease（AOSD）,we retrospectively analyzed 53 cases of AOSD.Method：We retrospectively analyzed the clinical data,auxiliary examination and prognosis of 53 patients with AOSD in our hospital.Result：In the retrospective analysis,women were more than men（52.8% vs.47.2%）,patients with severe complications such as multiple organ dysfunction and hemophagocytic syndrome had poor prognosis.There were no significant differences in baseline level of procalcitonin,interleukin-6,C reactive protein,erythrocyte sedimentation rate,white blood cell count and neutrophil percentage in patients with different prognosis.Ferritin levels of patients with good prognosis were significantly lower than in patients with poor prognosis after treatment[（4 898±8 938）μg/L vs.（31 810±17 710）μg/L,P〈0.001],ferritin levels of patients with good prognosis significantly decreased compared to baseline [（4 898±8 938）μg/L vs.（14 270±19 070）μg/L,P〈0.001],and ferritin levels continued to increase in patients with poor prognosis after treatment[（31 810±17 710）μg/L vs.（15 240±22 150）μg/L,P〈0.001].Conclusion：AOSD is a kind of disease characterized by fever,rash,joint pain.Ferritin has good predictive effect on the diagnosis and treatment of patients,most patients had a good prognosis,patients with serious complications had a poor prognosis.