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RMD Open. 2016 Apr 20;2(1):e000179. doi: 10.1136/rmdopen-2015-000179. eCollection 2016.

Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort.

Author information

Service de rhumatologie , CHRU Cavale Blanche, EA 2216, INSERM ERI 29, Université de Bretagne occidentale , Brest Cedex , France.
Service de rhumatologie , Hôpitaux Universitaires Paris-Sud, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris-Sud, INSERM U1184 , Le Kremlin-Bicêtre , France.
Service de rhumatologie , CHU Hautepierre , Strasbourg , France.
Service de rhumatologie , Centre hospitalier Le Mans , Le Mans Cedex , France.
Service de rhumatologie , CHU Gabriel Montpied , Clermont-Ferrand Cedex , France.
Service de rhumatologie , CHU Bois-Guillaume , Rouen Cedex , France.
Service de médecine interne , CHU Claude Huriez , Lille Cedex , France.
Service de médecine interne , Hôpital Avicenne APHP , Bobigny Cedex , France.
Centre de référence des maladies auto-immunes rares, CHU Cochin APHP , Paris , France.



Neurological manifestations seem common in primary Sjögren's syndrome (pSS) but their reported prevalences vary. We investigated the prevalence and epidemiology of neurological manifestations in a French nationwide multicentre prospective cohort of patients with pSS, the Assessment of Systemic Signs and Evolution in Sjögren's syndrome (ASSESS) cohort.


The ASSESS cohort, established in 2006, includes 395 patients fulfilling American-European Consensus Group criteria for pSS. Demographic and clinical data were compared between patient groups with and without neurological manifestations, and across patient groups with peripheral nervous system (PNS) manifestations, central nervous system (CNS) manifestations and no neurological manifestations.


Data at inclusion were available for 392 patients, whose mean age was 58±12 years. Mean follow-up was 33.9 months. Neurological manifestations were present in 74/392 (18.9%) patients, including 63 (16%) with PNS manifestations and 14 (3.6%) with CNS manifestations. Prevalences were 9.2% for pure sensory neuropathy, 5.3% for sensorimotor neuropathy, 1.3% for cerebral vasculitis and 1.0% for myelitis. Neurological manifestations were associated with greater pSS activity as assessed using the ESSDAI (9.4±6.8 vs 4.3±4.8; p<0.001) and proportion of patients taking immunomodulatory/immunosuppressive drugs (32.4% (24/74) versus 13.8% (44/318), p=0003). New neurological symptoms were more common in patients with than without prior neurological manifestations (RR=3.918 (95% CI 1.91 to 8.05); p<0.001).


Prevalences of peripheral and central neurological manifestations in pSS are about 15% and 5%, respectively. Neurological manifestations are associated with greater pSS activity. New neurological manifestations are more common in patients with prior neurological involvement.


Autoimmune Diseases; Disease Activity; Sjøgren's Syndrome

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