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干燥综合征和其他罕见且复杂的结缔组织疾病:一种有趣的关联。

Sjögren's syndrome and other rare and complex connective tissue diseases: an intriguing liaison.

机构信息

Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Pisa, Italy.

Department of Rheumatology, Hôpitaux Universitaires de Strasbourg, INSERM UMR-S 1109, Centre National de Référence des Maladies Auto-Immunes Systémiques Rares (RESO), Strasbourg, France.

出版信息

Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):103-112. doi: 10.55563/clinexprheumatol/3y0hqs. Epub 2022 Mar 21.

Abstract

Sjögren's syndrome (SS) is a systemic autoimmune disease that frequently occurs concomitantly with other systemic connective tissue disorders, including rare and complex diseases such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). The presence of SS influences the clinical expression of the other autoimmune diseases, thus offering the unique opportunity to explore the similarities in genetic signatures, as well as common environmental and biologic factors modulating the expression of disease phenotypes. In this review, we will specifically discuss the possibility of defining "SS/SLE" and "SS/SSc" as distinct subsets within the context of connective tissue diseases with different clinical expression and outcomes, thus deserving an individualised assessment and personalised medical interventions.

摘要

干燥综合征(SS)是一种系统性自身免疫性疾病,常与其他系统性结缔组织疾病同时发生,包括系统性红斑狼疮(SLE)和系统性硬皮病(SSc)等罕见且复杂的疾病。SS 的存在会影响其他自身免疫性疾病的临床表现,因此提供了探索遗传特征相似性以及调节疾病表型表达的共同环境和生物学因素的独特机会。在这篇综述中,我们将专门讨论在具有不同临床表现和结局的结缔组织疾病背景下,将“SS/SLE”和“SS/SSc”定义为不同亚组的可能性,因此需要进行个体化评估和个性化医疗干预。

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