干燥综合征合并周围神经病变的分类及特征
发表者：赵金霞
日期：2015-03-04
浏览次数：744
评论次数：0
摘要
目的：本研究旨在分析干燥综合征合并不同类型周围神经病的病因、临床特征以及预后，并探讨其与临床和免疫学指标的关系。
方法：连续入选563例确诊为原发性干燥综合征的患者。回顾性分析可疑周围神经系统受累患者的神经传导检查结果。根据神经电生理检查结果将周围神经病分为单神经病、多发性单神经病、多神经病和神经元病。
结果：158例（28%）干燥综合征患者进行了神经传导检查。49例结果正常，109例异常，其中102例诊断为周围神经病。排除因其他疾病引起的神经病变以及卡压性单神经病变，55例（10%）患者确诊为干燥综合征相关性周围神经病，包括轴突性感觉运动多神经病（n=24），感觉神经病（n=15），多发性单神经病（n=15）和脱髓鞘多发性神经根神经病（n=1）。尽管接受了治疗，仍有12%的轴突性多神经病，13%的多发性单神经病和47%的运动神经元病患者疾病进展。合并周围神经病（尤其是多发性单神经病和轴突性多神经病）患者的生存期远低于对照组。
结论：干燥综合征相关周围神经病的发生率为10%。根据临床表现和神经电生理诊断检查对周围神经病进行分类可能有助于判断功能状态，治疗反应以及生存情况。
附原文：Abstract OBJECTIVES: This paper aims to analyse the etiology, characterisation and outcomes of the different types of peripheral neuropathy in patients with primary Sjögren's syndrome (SS) and their association with clinical and immunological disease expression. METHODS: A total of 563 consecutive patients diagnosed with primary SS were evaluated. We retrospectively assessed the results of nerve conduction studies carried out in patients with suspected peripheral nervous system involvement. Peripheral neuropathies were classified into mononeuropathy, mononeuropathy multiplex, polyneuropathy and neuronopathy according to the patterns evidenced by electrodiagnostic studies. RESULTS: Nerve conduction studies were carried out in 158/563 (28%) SS patients. The results were normal in 49 and abnormal in 109 patients, in whom peripheral neuropathy was diagnosed in 102. After excluding patients with neuropathy associated with other diseases and patients with entrapment mononeuropathies, 55/563 (10%) patients were classified as having SS-related peripheral neuropathy, including axonal sensorimotor polyneuropathy (n=24), pure sensory neuronopathy (n=15), mononeuropathy multiplex (n=15) and demyelinating polyradiculoneuropathy (n=1). In spite of therapy, clinical progression measured by the MOHS scale was observed in 12% of patients with axonal polyneuropathy, 13% of those with mononeuropathy multiplex and 47% of those with neuronopathy. Survival was significantly reduced in patients with peripheral neuropathy (especially in those with mononeuropathy multiplex and axonal polyneuropathy) in comparison with the control group (log rank =0.001). CONCLUSIONS: We found a prevalence of SS-related peripheral neuropathy of 10%. Classification of neuropathy according to the clinical presentation and electrodiagnostic tests may be useful in determining the functional outcome, therapeutic response and survival.
引自: Brito-Zerón P, Akasbi M, Bosch X, Bové A, et al. Classification and characterisation of peripheral neuropathies in 102 patients with primary Sjögren's syndrome. Clin Exp Rheumatol. 2013 Jan-Feb;31(1):103-10. Epub 2012 Sep 27. （注：干燥综合症病人或亲属可加QQ群交流，群号： 118194945 ，本网站站长私人微信号： ssgzz88 ）
目的：本研究旨在分析干燥综合征合并不同类型周围神经病的病因、临床特征以及预后，并探讨其与临床和免疫学指标的关系。
方法：连续入选563例确诊为原发性干燥综合征的患者。回顾性分析可疑周围神经系统受累患者的神经传导检查结果。根据神经电生理检查结果将周围神经病分为单神经病、多发性单神经病、多神经病和神经元病。
结果：158例（28%）干燥综合征患者进行了神经传导检查。49例结果正常，109例异常，其中102例诊断为周围神经病。排除因其他疾病引起的神经病变以及卡压性单神经病变，55例（10%）患者确诊为干燥综合征相关性周围神经病，包括轴突性感觉运动多神经病（n=24），感觉神经病（n=15），多发性单神经病（n=15）和脱髓鞘多发性神经根神经病（n=1）。尽管接受了治疗，仍有12%的轴突性多神经病，13%的多发性单神经病和47%的运动神经元病患者疾病进展。合并周围神经病（尤其是多发性单神经病和轴突性多神经病）患者的生存期远低于对照组。
结论：干燥综合征相关周围神经病的发生率为10%。根据临床表现和神经电生理诊断检查对周围神经病进行分类可能有助于判断功能状态，治疗反应以及生存情况。
附原文：Abstract OBJECTIVES: This paper aims to analyse the etiology, characterisation and outcomes of the different types of peripheral neuropathy in patients with primary Sjögren's syndrome (SS) and their association with clinical and immunological disease expression. METHODS: A total of 563 consecutive patients diagnosed with primary SS were evaluated. We retrospectively assessed the results of nerve conduction studies carried out in patients with suspected peripheral nervous system involvement. Peripheral neuropathies were classified into mononeuropathy, mononeuropathy multiplex, polyneuropathy and neuronopathy according to the patterns evidenced by electrodiagnostic studies. RESULTS: Nerve conduction studies were carried out in 158/563 (28%) SS patients. The results were normal in 49 and abnormal in 109 patients, in whom peripheral neuropathy was diagnosed in 102. After excluding patients with neuropathy associated with other diseases and patients with entrapment mononeuropathies, 55/563 (10%) patients were classified as having SS-related peripheral neuropathy, including axonal sensorimotor polyneuropathy (n=24), pure sensory neuronopathy (n=15), mononeuropathy multiplex (n=15) and demyelinating polyradiculoneuropathy (n=1). In spite of therapy, clinical progression measured by the MOHS scale was observed in 12% of patients with axonal polyneuropathy, 13% of those with mononeuropathy multiplex and 47% of those with neuronopathy. Survival was significantly reduced in patients with peripheral neuropathy (especially in those with mononeuropathy multiplex and axonal polyneuropathy) in comparison with the control group (log rank =0.001). CONCLUSIONS: We found a prevalence of SS-related peripheral neuropathy of 10%. Classification of neuropathy according to the clinical presentation and electrodiagnostic tests may be useful in determining the functional outcome, therapeutic response and survival.
引自: Brito-Zerón P, Akasbi M, Bosch X, Bové A, et al. Classification and characterisation of peripheral neuropathies in 102 patients with primary Sjögren's syndrome. Clin Exp Rheumatol. 2013 Jan-Feb;31(1):103-10. Epub 2012 Sep 27. （注：干燥综合症病人或亲属可加QQ群交流，群号： 118194945 ，本网站站长私人微信号： ssgzz88 ）