Department of Pathophysiology, Medical School, University of Athens, Athens, Greece.
J Neurol Neurosurg Psychiatry. 2011 Jul;82(7):798-802. doi: 10.1136/jnnp.2010.222109. Epub 2010 Dec 16.
The prevalence of peripheral neuropathy in patients with Sjögren syndrome remains unclear owing to conflicting results in the published series, with numbers ranging from 2% to over 60% of Sjögren syndrome patients. Whether peripheral neuropathy is a feature of the systemic or glandular disease or whether it is related to a circulating antineuronal antibody remains also uncertain.
The authors reviewed the records of patients with primary Sjögren syndrome (pSS), fulfilling the Revised European-American Classification Criteria, seen in their department from 1992 to 2009. The patients with previously recorded neuropathic features were re-examined clinically and electrophysiologically. Other causes of polyneuropathy were excluded. The authors also searched for circulating antineural antibodies using immunofluorescence and western blot and for antibodies against muscarinic and nicotinic acetylcholine receptors as potential biomarkers.
509 cases met the diagnostic criteria for pSS. Among these, 44 patients were recorded as having neuropathic symptoms. After completing the evaluation, however, only nine (1.8%) had polyneuropathy with objective clinical signs and abnormal electrophysiological findings. The neuropathy was axonal in all, in five pure sensory and in four sensorimotor. The patients with peripheral neuropathy had extraglandular manifestations such as palpable purpura and vasculitis. No evidence of antineural autoimmunity was found, and no candidate biomarkers were identified.
Polyneuropathy is a rare manifestation of pSS occurring in 1.8% of patients. In the majority of patients, it is a late event and frequently associated with systemic disease or risk factors for lymphoma development.
由于发表的系列结果存在冲突，干燥综合征患者周围神经病变的患病率仍不清楚，范围从 2%到超过 60%的干燥综合征患者。周围神经病变是系统性疾病还是腺体疾病的特征，或者是否与循环抗神经元抗体有关，目前仍不确定。
作者回顾了 1992 年至 2009 年期间在其科室就诊的原发性干燥综合征（pSS）患者的记录，这些患者符合修订后的欧洲-美国分类标准。对有记录的神经病变特征的患者进行了临床和电生理复查。排除了其他原因引起的多发性神经病。作者还使用免疫荧光和免疫印迹法寻找循环抗神经抗体，并寻找毒蕈碱和烟碱型乙酰胆碱受体抗体作为潜在的生物标志物。
509 例符合 pSS 的诊断标准。其中，44 例患者记录有神经病变症状。然而，在完成评估后，仅有 9 例（1.8%）患者存在客观临床体征和异常电生理发现的多发性神经病。所有患者的神经病变均为轴索性，5 例为纯感觉性，4 例为感觉运动性。周围神经病患者有可触及性紫癜和血管炎等腺体外表现。未发现抗神经自身免疫证据，也未发现候选生物标志物。
多发性神经病是 pSS 的罕见表现，在 1.8%的患者中发生。在大多数患者中，它是一种晚期事件，常与系统性疾病或淋巴瘤发展的危险因素有关。
