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干燥综合征。

Sjögren's syndrome.

机构信息

Department of Dermatology, Venereology and Allergology, Medical University of Innsbruck, Innsbruck, Austria.

出版信息

J Dtsch Dermatol Ges. 2022 Jul;20(7):980-1002. doi: 10.1111/ddg.14823. Epub 2022 Jul 1.

Abstract

Sjögren's syndrome (SjS) is an autoimmune disease characterized by the triad of sicca symptoms, fatigue and pain. This diagnosis is usually made in women at the average age of 60 years. Diagnosis is made when sicca symptoms persist for more than three months, after the exclusion of possible differential diagnoses, and using the ACR/EULAR 2016 classification criteria for SjS. Many organs can be affected in the course of this disease. Xerosis cutis and pruritus are the most common skin manifestations, followed by leukocytoclastic vasculitis and subacute cutaneous lupus erythematosus. In addition, SjS patients often have myoarthralgia and neuropsychiatric symptoms. In the long term, attention must be paid to the increased risk of cardiovascular disease and lymphoma. Due to the multiorgan involvement in SjS patients, interdisciplinary care is required.

摘要

干燥综合征(SjS)是一种自身免疫性疾病,其特征为干燥症状、疲劳和疼痛三联征。这种诊断通常在女性平均年龄为 60 岁时做出。当干燥症状持续超过三个月,排除可能的鉴别诊断后,并使用 ACR/EULAR 2016 干燥综合征分类标准时,即可做出诊断。在疾病过程中,许多器官都可能受到影响。皮肤干燥和瘙痒是最常见的皮肤表现,其次是白细胞碎裂性血管炎和亚急性皮肤型狼疮。此外,SjS 患者常伴有肌痛和神经精神症状。长期来看,应注意心血管疾病和淋巴瘤的风险增加。由于 SjS 患者多器官受累,需要多学科治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e539/9539881/efa1e4890040/DDG-20-980-g001.jpg

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