作者：Guillermo Carvajal Alegria 译者：姚中强 校者：孙琳

目的:原发性干燥综合征(pSS)的神经系统表现似乎常见，但其发生率报道不一。我们研究了一个法国全国性多中心前瞻性队列-干燥综合征系统体征和演变评估 (ASSESS)中神经系统表现的发生率。

方法:ASSESS队列始于2006年，包括395名符合美国-欧洲共识标准的干燥综合征患者。比较了有无神经系统表现的患者人口学和临床数据的不同，也比较了有周围神经系统(PNS)表现、中枢神经系统(CNS)表现和无神经系统表现者。

结果:392名纳入患者有数据，平均年龄为58±12?岁，平均随访时间为 33.9月。 神经系统表现见于74/392 (18.9%) 患者, 包括 63 (16%)周围神经系统表现和 14 (3.6%) 中枢神经系统表现。其中9.2% 纯感觉神经病变, 5.3% 感觉-运动神经病变, 1.3% 颅脑血管炎和1.0% 脊髓炎。 有神经系统表现的pSS患者ESSDAI 病情活动性更高 (9.4±6.8 vs 4.3±4.8; p<0 .001)，使用免疫调节剂/免疫抑制剂药物的比例更高(32.4% (24/74) vs 13.8% (44/318), p="0003)。" 新发神经系统表现更多见于既往有神经系统表现者 (rr="3.918" (95% ci 1.91 to 8.05); p<0.001)。

结论:原发性干燥综合征患者周围和中枢神经系统受累率分别为15% 和 5%。 神经系统表现与更高的病情活动性相关。新发神经系统表现更常见于既往神经系统受累者。

附原文：Objectives Neurological manifestations seem common in primary Sj?gren'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?gren'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.

引自：Guillermo Carvajal Alegria, Dewi Guellec, Xavier Mariette, Jacques-Eric Gottenberg, Emmanuelle Dernis, Jean-Jacques Dubost, Anne-Priscille Trouvin, Eric Hachulla, Claire Larroche, Veronique Le Guern, Divi Cornec, Valérie Devauchelle-Pensec, and Alain Saraux, on behalf of the Assessment of Systemic Signs and Evolution in Sj?gren's Syndrome (ASSESS) group. Epidemiology of neurological manifestations in Sj?gren's syndrome: data from the French ASSESS Cohort. RMD Open. 2016; 2(1): e000179.