56例成人still病临床特征分析

Analysis on clinical characteristics of 56 cases with adult-onset Still’s disease

目的分析成人still病(AOSD)患者的临床特征、实验室指标及治疗转归的特点,为临床诊治提供经验。方法回顾性分析56例AOSD患者临床资料,并对其进行临床特征分析。结果AOSD以发热、皮疹、关节痛、咽痛起病,伴有肝损、肌肉酸痛、淋巴结肿大、脾大。实验室检查中白细胞、中性粒细胞、炎性指标、血清铁蛋白明显升高。非甾体抗炎药治疗效果不明显,所有患者均使用了糖皮质激素,19例联合使用甲氨蝶呤,9例联合使用硫酸羟氯喹,7例联合使用环孢素,1例联合使用环磷酰胺。11例难治性AOSD患者中有9例使用托珠单抗,有2例合并巨噬细胞活化综合征(MAS),其中1例好转,1例最终死于重症感染。结论AOSD主要临床特征为发热、皮疹、关节痛、浅表淋巴结肿大、咽痛、脾大,目前仍缺乏特异性实验室指标,血清铁蛋白对患者的诊断和治疗具有较好的预测效果,其主要治疗为糖皮质激素及免疫抑制剂,病程中应警惕MAS可能,对难治性AOSD可以使用托珠单抗治疗。

Objective To analyze the clinical characteristics,laboratory indicators and treatment outcome of patients with adult-onset Still’s disease(AOSD)and to provide experience for clinical diagnosis and treatment.Methods The clinical data of 56 cases with AOSD were retrospectively analyzed and their clinical characteristics were analyzed.Results AOSD patients started with fever,rash,joint pain and pharyngeal pain,accompanied by liver damage,muscle pain,lymph node enlargement and splenomegaly.In laboratory examination,leukocytes,neutrophils,inflammatory markers and serum ferritin were significantly increased.The effect of NSAIDs was not obvious.Glucocorticoid was used in all patients,19 cases were combined with methotrexate,9 cases were combined with hydroxychloroquine sulfate,7 cases were combined with cyclosporin,and one case was combined with cyclophosphamide.Tocilizumab was used in 9 of 11 patients with refractory AOSD,two cases were associated with macrophage activation syndrome(MAS),one case was improved and one case was eventually died of severe infection.Conclusion The main clinical features of AOSD are fever,rash,joint pain,superficial lymph node enlargement,sore throat and splenomegaly.There is still lack of specificity laboratory index,serum ferritin in patients with the diagnosis and treatment which has a good prediction effect.Glucocorticoids and immunosuppressive agents are the main treatment methods,the possibility of MAS should be alert in the course of the disease.