RT Journal Article
SR Electronic
T1 Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort
JF RMD Open
JO RMD Open
FD EULAR
SP e000179
DO 10.1136/rmdopen-2015-000179
VO 2
IS 1
A1 Guillermo Carvajal Alegria
A1 Dewi Guellec
A1 Xavier Mariette
A1 Jacques-Eric Gottenberg
A1 Emmanuelle Dernis
A1 Jean-Jacques Dubost
A1 Anne-Priscille Trouvin
A1 Eric Hachulla
A1 Claire Larroche
A1 Veronique Le Guern
A1 Divi Cornec
A1 Valérie Devauchelle-Pensec
A1 Alain Saraux
YR 2016
UL http://rmdopen.bmj.com/content/2/1/e000179.abstract
AB Objectives 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.Methods 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.Results 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).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.