• Rev Med Interne · Aug 2023

    Review

    French national diagnostic and care protocol for Sjögren's syndrome.

    • Valérie Devauchelle-Pensec, Xavier Mariette, Anas-Alexis Benyoussef, Sylvie Boisrame, Béatrice Cochener, Divi Cornec, Gaëtane Nocturne, Jacques Eric Gottenberg, Eric Hachulla, Pierre Labalette, Véronique Le Guern, Ruth M'Bwang Seppoh, Jacques Morel, Marie Orliaguet, Alain Saraux, Raphaèle Seror, Nathalie Costedoat-Chalumeau, and Collaborators.
    • Service de Rhumatologie, CHU de Brest, Inserm 1227, LBAI, Université de Bretagne Occidentale, Centre de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), 29609 Brest cedex, France. Electronic address: valerie.devauchelle-pensec@chu-brest.fr.
    • Rev Med Interne. 2023 Aug 1; 44 (8): 423457423-457.

    AbstractSjögren's disease (SD), also known as Sjögren's syndrome (SS) or Gougerot-Sjögren's syndrome in France, is a rare systemic autoimmune disease in its primary form and is characterised by tropism for the exocrine glandular epithelia, particularly the salivary and lacrimal glands. The lymphocytic infiltration of these epithelia will clinically translate into a dry syndrome which, associated with fatigue and pain, constitutes the symptom triad of the disease. In about one third of patients, SD is associated with systemic complications that can affect the joints, skin, lungs, kidneys, central or peripheral nervous system, and lymphoid organs with an increased risk of B-cell lymphoma. SD affects women more frequently than men (9/1). The peak frequency is around the age of 50. However, the disease can occur at any age, with paediatric forms occurring even though they remain rare. SD can occur alone or in association with other systemic autoimmune diseases. In its isolated or primary form, the prevalence of SD is estimated to be between 1 per 1000 and 1 per 10,000 inhabitants. The most recent classification criteria were developed in 2016 by EULAR and ACR. The course and prognosis of the disease are highly variable and depend on the presence of systemic involvement and the severity of the dryness of the eyes and mouth. The current approach is therefore to identify at an early stage those patients most at risk of systemic complications or lymphoma, who require close follow-up. On the other hand, regular monitoring of the ophthalmological damage and of the dental status should be ensured to reduce the consequences.Copyright © 2023. Published by Elsevier Masson SAS.

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