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成人斯蒂尔病女性患者病例报告及文献复习。

Case Report of a Female Patient with Adult-onset Still's Disease and Review of The Literature.

机构信息

Department of Pharmacy, Jiading District Hospital of Traditional Chinese Medicine, Shanghai 201800, China.

出版信息

Iran J Immunol. 2022 Jun;19(2):207-212. doi: 10.22034/iji.2022.92228.2137.

Abstract

BACKGROUND

Adult-onset Still's disease (AOSD), which presents many non-specific symptoms, such as rash leukocytosis, spiking fever, and sore throat, is a rare auto inflammatory disease. Other clinical features that are frequently observed include lymphadenopathy, arthralgia, serositis, splenomegaly, and hepatomegaly. Laboratory tests show high levels of C-reactive protein, ferritin, and erythrocyte sedimentation rate reflecting the systemic inflammatory process in AOSD patients.

CASE PRESENTATION

The patient was a middle-aged woman with a high fever (39.8 C), sore throat, rashes on limbs with pruritus, mainly at the joints (elbow, knee, and ankle), muscle aches, dizziness, infirmity, weakness, and poor appetite without arthralgia. The ferritin level was above 1500 (normal value: 14-233) ng/L. Antineutrophil, antinuclear antibodies, and rheumatoid factor were negative. Combining the symptoms such as fever, rash, stress-induced acute inflammation, arthritis, and ferritin levels, the patient was eventually diagnosed with adult Still's disease. She received methylprednisolone 40mg intravenously every 12 hours for one week. On the second week, the dose was reduced to 40mg in the morning and 20mg in the evening, and finally, the dose was reduced to 40mg oral intake in the morning and 8mg in the evening. After half a month of treatment, the patient's high fever and skin rashes subsided, and the other symptoms also gradually relieved.

CONCLUSIONS

A case of a middle-aged woman diagnosed with adult Still's disease is reported, and the possible pathogenesis and treatment of the disease are discussed. This case highlights the importance of early diagnosis and timely treatment of adult Still's disease to prevent potentially fatal complications.

摘要

背景

成人斯蒂尔病(AOSD)是一种罕见的自身炎症性疾病,表现为皮疹、白细胞增多、高热、咽痛等许多非特异性症状。其他常观察到的临床特征包括淋巴结病、关节炎、浆膜炎、脾肿大和肝肿大。实验室检查显示 C 反应蛋白、铁蛋白和红细胞沉降率升高,反映 AOSD 患者的全身炎症过程。

病例介绍

患者为中年女性,高热(39.8°C),咽痛,四肢皮疹伴瘙痒,主要累及关节(肘、膝和踝),肌肉疼痛,头晕,虚弱,乏力,食欲不振,无关节炎。铁蛋白水平高于 1500(正常值:14-233)ng/L。抗中性粒细胞、抗核抗体和类风湿因子均为阴性。结合发热、皮疹、应激诱导的急性炎症、关节炎和铁蛋白水平等症状,最终诊断为成人斯蒂尔病。患者接受甲基强的松龙 40mg 静脉注射,每 12 小时一次,共 1 周。第 2 周,剂量减为上午 40mg,晚上 20mg,最后,剂量减为早上 40mg 口服,晚上 8mg 口服。治疗半个月后,患者高热和皮疹消退,其他症状也逐渐缓解。

结论

报道了一例中年女性成人斯蒂尔病的病例,并讨论了该病的可能发病机制和治疗方法。该病例强调了早期诊断和及时治疗成人斯蒂尔病的重要性,以预防潜在的致命并发症。

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