免疫球蛋白对原发干燥综合征相关神经病变的疗效随类型不同而不同
发表者：张颖健
日期：2015-03-07
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摘要（法国） 目的：干燥综合征（SS）相关的外周神经病可致残且尚无有效治疗措施。一些个案报道静脉输注免疫球蛋白（IVIG）可使患者获益。本研究旨在探明IVIG治疗SS相关外周神经病的有效性。
方法：我们对一项多中心回顾性研究中的19例无坏死性血管炎的pSS相关神经病应用IVIG治疗的效果和耐受度进行评估。应用改良Rankin量表（MRS）和医师总体评估对疗效反应进行评价。
结果：8例患者（42%）MRS得分降低并与临床改善相关，10例（52%）MRS得分稳定，1例（6%）MRS得分增加。在医师总体评估中，9例（47%）得到改善，6例（31%）稳定，4例（21%）恶化。所有感觉运动型（n=5）或非共济失调性感觉型神经病（n=4）得到改善或稳定。然而，患有共济失调性神经病（n=9）的患者中仅2例改善，4例恶化。给予IVIG后，13例应用糖皮质激素治疗的患者中有10例可将泼尼松剂量从15mg/d降至10mg/d。仅1例患者由于轻微副作用和缺乏初始有效性在应用一次IVIG后停用。
结论：IVIG对治疗SS相关无坏死性血管炎的感觉运动型神经病或非共济失调性感觉型神经病可能有效。然而对治疗SS相关共济失调性神经病的效果不清楚。
附原文：Abstract OBJECTIVE: Sjögren's syndrome (SS)-related peripheral neuropathy is responsible for disability, but no treatment has been shown to improve its outcome. In some cases, intravenous immunoglobulin (IVIG) therapy has been associated with some benefit. In this study, we investigated the effectiveness of IVIG in SS-related peripheral neuropathy.METHODS: We assessed the efficacy and tolerance of IVIG in 19 patients with primary SS-related neuropathy without any necrotizing vasculitis in a retrospective national multicentric study. The evaluation of the response was assessed using the disability Modified Rankin Scale (MRS) and a global evaluation by the practitioner.RESULTS: Eight patients (42%) exhibited a decrease of the MRS score corresponding to a clinical improvement, 10 patients (52%) exhibited a stable MRS score, and 1 patient (6%) showed an increase of MRS score. According to the global evaluation by the practitioner, 9 (47%) of the 19 patients were improved, 6 patients (31%) were stable, and 4 patients (21%) worsened. All the patients with sensorimotor (n = 5) or nonataxic sensory neuropathy (n = 4) were improved or stabilized. However, among the patients with ataxic neuropathy (n = 9), only 2 improved and 4 worsened. Ten of the 13 patients treated with corticosteroids could have had the prednisone dosage decreased from 15 mg/day (range 7.5-60) to 10 mg/day (range 5-20) with IVIG. Only 1 patient stopped the treatment after 1 dose because of a minor side effect and lack of initial efficacy.CONCLUSION: IVIG may be useful in the treatment of SS-associated sensorimotor neuropathies or nonataxic sensory neuropathy without any necrotizing vasculitis. The benefit of such therapy in the SS-related ataxic neuropathy seems less clear.
引自：Rist S, Sellam J, Hachulla E, Sordet C, Puéchal X, Hatron PY, Benhamou CL, Sibilia J, Mariette X; Club Rhumatismes et Inflammation. Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren's syndrome: a national multicentric retrospective study. Arthritis Care Res (Hoboken). 2011 Sep;63(9):1339-44. doi: 10.1002/acr.20495. （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 ）
方法：我们对一项多中心回顾性研究中的19例无坏死性血管炎的pSS相关神经病应用IVIG治疗的效果和耐受度进行评估。应用改良Rankin量表（MRS）和医师总体评估对疗效反应进行评价。
结果：8例患者（42%）MRS得分降低并与临床改善相关，10例（52%）MRS得分稳定，1例（6%）MRS得分增加。在医师总体评估中，9例（47%）得到改善，6例（31%）稳定，4例（21%）恶化。所有感觉运动型（n=5）或非共济失调性感觉型神经病（n=4）得到改善或稳定。然而，患有共济失调性神经病（n=9）的患者中仅2例改善，4例恶化。给予IVIG后，13例应用糖皮质激素治疗的患者中有10例可将泼尼松剂量从15mg/d降至10mg/d。仅1例患者由于轻微副作用和缺乏初始有效性在应用一次IVIG后停用。
结论：IVIG对治疗SS相关无坏死性血管炎的感觉运动型神经病或非共济失调性感觉型神经病可能有效。然而对治疗SS相关共济失调性神经病的效果不清楚。
附原文：Abstract OBJECTIVE: Sjögren's syndrome (SS)-related peripheral neuropathy is responsible for disability, but no treatment has been shown to improve its outcome. In some cases, intravenous immunoglobulin (IVIG) therapy has been associated with some benefit. In this study, we investigated the effectiveness of IVIG in SS-related peripheral neuropathy.METHODS: We assessed the efficacy and tolerance of IVIG in 19 patients with primary SS-related neuropathy without any necrotizing vasculitis in a retrospective national multicentric study. The evaluation of the response was assessed using the disability Modified Rankin Scale (MRS) and a global evaluation by the practitioner.RESULTS: Eight patients (42%) exhibited a decrease of the MRS score corresponding to a clinical improvement, 10 patients (52%) exhibited a stable MRS score, and 1 patient (6%) showed an increase of MRS score. According to the global evaluation by the practitioner, 9 (47%) of the 19 patients were improved, 6 patients (31%) were stable, and 4 patients (21%) worsened. All the patients with sensorimotor (n = 5) or nonataxic sensory neuropathy (n = 4) were improved or stabilized. However, among the patients with ataxic neuropathy (n = 9), only 2 improved and 4 worsened. Ten of the 13 patients treated with corticosteroids could have had the prednisone dosage decreased from 15 mg/day (range 7.5-60) to 10 mg/day (range 5-20) with IVIG. Only 1 patient stopped the treatment after 1 dose because of a minor side effect and lack of initial efficacy.CONCLUSION: IVIG may be useful in the treatment of SS-associated sensorimotor neuropathies or nonataxic sensory neuropathy without any necrotizing vasculitis. The benefit of such therapy in the SS-related ataxic neuropathy seems less clear.
引自：Rist S, Sellam J, Hachulla E, Sordet C, Puéchal X, Hatron PY, Benhamou CL, Sibilia J, Mariette X; Club Rhumatismes et Inflammation. Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren's syndrome: a national multicentric retrospective study. Arthritis Care Res (Hoboken). 2011 Sep;63(9):1339-44. doi: 10.1002/acr.20495. （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 ）