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Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy

1
Laboratory of Pathophysiological and Nutritional Biochemistry, Université Libre de Bruxelles, 1070 Brussels, Belgium
2
Department of Rheumatology, Erasme Hospital, Université Libre de Bruxelles, 1070 Brussels, Belgium
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2020, 9(7), 2299; https://doi.org/10.3390/jcm9072299
Received: 25 June 2020 / Revised: 15 July 2020 / Accepted: 16 July 2020 / Published: 20 July 2020
(This article belongs to the Special Issue Diseases of the Salivary Glands)
Primary Sjögren’s syndrome (pSS) is a chronic systemic autoimmune rheumatic disease characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands, whereby sicca syndrome and/or systemic manifestations are the clinical hallmarks, associated with a particular autoantibody profile. pSS is the most frequent connective tissue disease after rheumatoid arthritis, affecting 0.3–3% of the population. Women are more prone to develop pSS than men, with a sex ratio of 9:1. Considered in the past as innocent collateral passive victims of autoimmunity, the epithelial cells of the salivary glands are now known to play an active role in the pathogenesis of the disease. The aetiology of the “autoimmune epithelitis” still remains unknown, but certainly involves genetic, environmental and hormonal factors. Later during the disease evolution, the subsequent chronic activation of B cells can lead to the development of systemic manifestations or non-Hodgkin’s lymphoma. The aim of the present comprehensive review is to provide the current state of knowledge on pSS. The review addresses the clinical manifestations and complications of the disease, the diagnostic workup, the pathogenic mechanisms and the therapeutic approaches. View Full-Text
Keywords: Sjögren’s syndrome; autoimmune disease; physiopathology; treatment; diagnosis; review Sjögren’s syndrome; autoimmune disease; physiopathology; treatment; diagnosis; review
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MDPI and ACS Style

Parisis, D.; Chivasso, C.; Perret, J.; Soyfoo, M.S.; Delporte, C. Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy. J. Clin. Med. 2020, 9, 2299. https://doi.org/10.3390/jcm9072299

AMA Style

Parisis D, Chivasso C, Perret J, Soyfoo MS, Delporte C. Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy. Journal of Clinical Medicine. 2020; 9(7):2299. https://doi.org/10.3390/jcm9072299

Chicago/Turabian Style

Parisis, Dorian; Chivasso, Clara; Perret, Jason; Soyfoo, Muhammad S.; Delporte, Christine. 2020. "Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy" J. Clin. Med. 9, no. 7: 2299. https://doi.org/10.3390/jcm9072299

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