Back to Journals » Open Access Rheumatology: Research and Reviews » Volume 11

Sjögren's syndrome and systemic lupus erythematosus: links and risks

Authors Pasoto SG, Adriano de Oliveira Martins V, Bonfa E

Received 31 October 2018

Accepted for publication 12 December 2018

Published 29 January 2019 Volume 2019:11 Pages 33—45

DOI https://doi.org/10.2147/OARRR.S167783

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Andrew Yee

Peer reviewer comments 2

Editor who approved publication: Professor Chuan-Ju Liu


Sandra Gofinet Pasoto,1,2 Victor Adriano de Oliveira Martins,1 Eloisa Bonfa1

1Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Sao Paulo, Brazil; 2Laboratory Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Sao Paulo, Brazil

Abstract: Systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SS) may coexist, and they are chronic complex disorders, with an autoimmune background, multifactorial etiology, multiple circulating autoantibodies, and variable prognosis. The prominent feature of SS is the impairment of the lacrimal and salivary glands leading to sicca symptoms. This disease may be classified as primary Sjögren’s syndrome (pSS), or secondary Sjögren’s syndrome (sSS) since it is often associated to other autoimmune disorders, principally SLE, rheumatoid arthritis, and systemic sclerosis. Systematic reviews and meta-analyses show an sSS prevalence in SLE patients of about 14%–17.8%. Herein, we updated important aspects of the clinical association between SLE and sSS through a narrative review of the PubMed database in the last 5 years (from July 2013 to October 2018) with the terms “Sjogren syndrome and systemic lupus erythematosus”. The following aspects are addressed: the classification criteria for sSS; differences and similarities between SLE and pSS regarding demographic, clinical, and serological characteristics (including new autoantibodies), as well as comorbidities; the etiopathogenic links between SLE and pSS (including genetic and environmental factors, B-cell activation, and autoantibodies); the predictive factors for sSS onset in SLE patients; the ocular and oral involvements due to sSS in SLE; and the main distinctive demographic, clinical, and serological features of SLE with and without associated SS.

Keywords: systemic lupus erythematosus, primary Sjögren’s syndrome, secondary Sjögren’s syndrome, polyautoimmunity, anti-Ro, anti-SSA, autoantibodies, pathogenesis

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]