@article {Carvajal Alegriae000179, author = {Guillermo Carvajal Alegria and Dewi Guellec and Xavier Mariette and Jacques-Eric Gottenberg and Emmanuelle Dernis and Jean-Jacques Dubost and Anne-Priscille Trouvin and Eric Hachulla and Claire Larroche and Veronique Le Guern and Divi Cornec and Val{\'e}rie Devauchelle-Pensec and Alain Saraux}, title = {Epidemiology of neurological manifestations in Sj{\"o}gren{\textquoteright}s syndrome: data from the French ASSESS Cohort}, volume = {2}, number = {1}, elocation-id = {e000179}, year = {2016}, doi = {10.1136/rmdopen-2015-000179}, publisher = {BMJ Specialist Journals}, abstract = {Objectives Neurological manifestations seem common in primary Sj{\"o}gren{\textquoteright}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 Sj{\"o}gren{\textquoteright}s syndrome (ASSESS) cohort.Methods The ASSESS cohort, established in 2006, includes 395 patients fulfilling American{\textendash}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.Results Data at inclusion were available for 392 patients, whose mean age was 58{\textpm}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{\textpm}6.8 vs 4.3{\textpm}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).Conclusions 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.}, URL = {https://rmdopen.bmj.com/content/2/1/e000179}, eprint = {https://rmdopen.bmj.com/content/2/1/e000179.full.pdf}, journal = {RMD Open} }