2022•Clinical and Experimental RheumatologyOpen access

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

Chiara Baldini, Laurent Arnaud, Tadej Avčin, Lorenzo Beretta, Chiara Bellocchi, Coralie Bouillot, Gerd‐Rüdiger Burmester, Lorenzo Cavagna, Maurizio Cutolo, Jeska K de Vries‐Bouwstra, Andrea. Schwartz Doria, Francesco Ferro, João Eurico Fonseca, Silvia Fonzetti, Giovanni Fulvio, Ilaria Galetti, Jacques‐Eric Gottenberg, Eric HACHULLA, Thomas M. Krieg, Gaetano La Rocca, Thierry Martin, Marco Matucci-Cerinic, Pia Moinzadeh, Carlomaurizio Montecucco, Marta Mosca, Luc Mouthon, U. Müller-Ladner, Simona Rednic, Vanessa M. Smith, Rosaria Talarico, Jacob M. van Laar, Ana Vieira, Vasco C. Romão, Xavier Mariette

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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.

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What this paper is about

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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Available 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.

Key concepts: Medicine, Connective tissue disease, Connective tissue, Systemic disease, Context (archaeology), Autoimmune disease, Immunology, Lupus erythematosus

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