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

Clin Exp Rheumatol

May 2022

Université Paris-Saclay, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, INSERM UMR1184, Reference Centre for Rare Systemic Autoimmube Diseases, Le Kremlin Bicêtre, France.

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.

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