International Journal of Medical Sciences

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20 October 2017

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Int J Med Sci 2017; 14(3):191-200. doi:10.7150/ijms.17718

Review

Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment

Tim Both1, Virgil A.S.H. Dalm1,2, P. Martin van Hagen1,2, Paul L.A. van Daele1,2✉

1. Department of Internal Medicine, Division of Clinical Immunology;
2. Department of Immunology, Erasmus MC, Rotterdam, The Netherlands.

Abstract

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This process leads to sicca syndrome, which is the combination of dryness of the eyes, oral cavity, pharynx, larynx and/or vagina. Extraglandular manifestations may also be prevalent in patients with pSS, including cutaneous, musculoskeletal, pulmonary, renal, hematological and neurological involvement. The pathogenesis of pSS is currently not well understood, but increased activation of B cells followed by immune complex formation and autoantibody production are thought to play important roles. pSS is diagnosed using the American-European consensus group (AECG) classification criteria which include subjective symptoms and objective tests such as histopathology and serology. The treatment of pSS warrants an organ based approach, for which local treatment (teardrops, moistures) and systemic therapy (including non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, disease-modifying antirheumatic drugs (DMARDS) and biologicals) can be considered. Biologicals used in the treatment of pSS mainly affect the total numbers of B cells (B cell depletion (Rituximab)) or target proteins required for B cell proliferation and/or activation (e.g. B cell activating factor (BAFF)) resulting in decreased B cell activity.

The aim of this review is to provide physicians a general overview concerning the pathogenesis, diagnosis and management of pSS patients.

Keywords: primary Sjögren syndrome, sicca, extraglandular syndrome, biologicals, epidemiology, pathogenesis, T-cell, B-cell.

This is an open access article distributed under the terms of the Creative Commons Attribution (CC BY-NC) License. See http://ivyspring.com/terms for full terms and conditions.
How to cite this article:
Both T, Dalm VASH, van Hagen PM, van Daele PLA. Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment. Int J Med Sci 2017; 14(3):191-200. doi:10.7150/ijms.17718. Available from http://www.medsci.org/v14p0191.htm