Abstract：
Primary Sjögren syndrome (pSS) is a chronic, systemic, autoimmune disorder characterized by lymphocytic infiltrates of the exocrine organs, leading to sicca symptoms and parotid enlargement. pSS has been linked to various neurological manifestations, including peripheral neuropathy (PN). We aimed to provide a comprehensive analysis of the currently available evidence regarding pSS-related PN.
A literature search in the PubMed database was performed, and 49 papers were eligible to be included in this systematic review and meta-analysis.
The pooled prevalence of PN in pSS is estimated to be 15.0% (95% confidence interval = 10.7%-20.7%). The mean age of pSS patients at PN diagnosis is 59 years. Among the patients with pSS and PN, 83% are females. Neuropathic symptoms usually precede or lead to the pSS diagnosis at a 2:1 ratio in patients with pSS-related PN. The commonest type of pSS-related PN is distal axonal polyneuropathy (80% of patients with pSS-related PN), followed by sensory ganglionopathy. Peripheral and cranial mononeuropathies-particularly trigeminal-are also frequent. Risk factors for developing PN include increasing age and presence of vasculitis. Immune-mediated pathogenetic mechanisms are discussed. Glucocorticoids are the most commonly used treatment option for managing pSS-related PN, when associated with vasculitis, followed by the use of intravenous immunoglobulin.
PN is very common in pSS patients. Evidence on long-term prognosis of PN in pSS is limited, and further research is needed. Research into the use of immunosuppressive medication in nonvasculitic neuropathies in the context of pSS merits further consideration.
A literature search in the PubMed database was performed, and 49 papers were eligible to be included in this systematic review and meta-analysis.
The pooled prevalence of PN in pSS is estimated to be 15.0% (95% confidence interval = 10.7%-20.7%). The mean age of pSS patients at PN diagnosis is 59 years. Among the patients with pSS and PN, 83% are females. Neuropathic symptoms usually precede or lead to the pSS diagnosis at a 2:1 ratio in patients with pSS-related PN. The commonest type of pSS-related PN is distal axonal polyneuropathy (80% of patients with pSS-related PN), followed by sensory ganglionopathy. Peripheral and cranial mononeuropathies-particularly trigeminal-are also frequent. Risk factors for developing PN include increasing age and presence of vasculitis. Immune-mediated pathogenetic mechanisms are discussed. Glucocorticoids are the most commonly used treatment option for managing pSS-related PN, when associated with vasculitis, followed by the use of intravenous immunoglobulin.
PN is very common in pSS patients. Evidence on long-term prognosis of PN in pSS is limited, and further research is needed. Research into the use of immunosuppressive medication in nonvasculitic neuropathies in the context of pSS merits further consideration.
摘要：
目的：原发性干燥综合征（pSS）是一种慢性，系统性，以外分泌器官的淋巴细胞浸润为特征的自身免疫性疾病，导致干燥症状和腮腺肿大。pSS与各种神经系统表现有关，包括周围神经病变（PN）。我们旨在提供有关pSS相关PN的现有证据的全面分析。
方法：在PubMed数据库中进行了文献检索，49篇论文符合纳入本系统综述和荟萃分析的条件.
结果：pSS中PN的合并患病率估计为15.0%（95%置信区间=10.7%-20.7%）。pSS患者在PN诊断时的平均年龄为59岁。在pSS和PN患者中，83%是女性。在pSS相关的PN患者中，神经性症状通常以2：1的比例先于或导致pSS诊断。最常见的pSS相关PN类型是远端轴索型多发性神经病（80%的pSS相关PN患者），其次是感觉神经节病变.周围性和头颅单神经病变-特别是三叉神经-也很常见。发生PN的危险因素包括年龄增加和血管炎的存在。讨论了免疫介导的致病机制。糖皮质激素是治疗pSS相关PN最常用的治疗选择，当与血管炎有关时，其次是使用静脉注射免疫球蛋白。
结论：PN在pSS患者中非常常见。关于pSS中PN长期预后的证据有限，需要进一步的研究。在pSS背景下，在非血管神经病中使用免疫抑制药物的研究值得进一步考虑。
方法：在PubMed数据库中进行了文献检索，49篇论文符合纳入本系统综述和荟萃分析的条件.
结果：pSS中PN的合并患病率估计为15.0%（95%置信区间=10.7%-20.7%）。pSS患者在PN诊断时的平均年龄为59岁。在pSS和PN患者中，83%是女性。在pSS相关的PN患者中，神经性症状通常以2：1的比例先于或导致pSS诊断。最常见的pSS相关PN类型是远端轴索型多发性神经病（80%的pSS相关PN患者），其次是感觉神经节病变.周围性和头颅单神经病变-特别是三叉神经-也很常见。发生PN的危险因素包括年龄增加和血管炎的存在。讨论了免疫介导的致病机制。糖皮质激素是治疗pSS相关PN最常用的治疗选择，当与血管炎有关时，其次是使用静脉注射免疫球蛋白。
结论：PN在pSS患者中非常常见。关于pSS中PN长期预后的证据有限，需要进一步的研究。在pSS背景下，在非血管神经病中使用免疫抑制药物的研究值得进一步考虑。
