Peripheral neuropathy associated with sicca complex
Peripheral neuropathy associated with sicca complex
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DOI：
10.1212/wnl.48.4.855
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发表时间：
1997-04-01
期刊：
影响因子：
9.9
通讯作者：
Dyck, PJ
中科院分区：
文献类型：
--
作者：
Grant, IA;Hunder, GG;Dyck, PJ
周围神经病变发生在干燥综合征中，干燥综合征是一种常见的全身免疫现象，包括血管炎。神经病变也可发生于孤立性干燥综合征（干燥性角结膜炎和口干）;这是否代表一种独特的综合征尚不清楚。我们回顾性研究了54例干燥综合征和周围神经病患者，以确定其表现方式、神经病理模式、血清学异常的频率和模式，以及包括坏死性血管炎在内的全身性疾病的频率。周围神经病变是87%的主要问题。虽然93%的患者出现干燥症状，但仅11%的患者主诉干燥症状，且症状通常较轻，仅在具体询问后报告。小涎腺活检阳性率为73%。感觉神经病占主导地位，61%的患者表现为感觉性多神经病或多神经节病。较少见的类型包括感觉运动性多发性神经病（17%）和多发性神经根神经病（11%）。血管炎性神经病变仅在两名患者中被证实，但70%的神经活检中存在非特异性神经外膜炎症。全身性疾病的临床证据是罕见的，特别是在感觉性多神经节病组，其中12例患者中只有1例发生体重减轻以外的腺体外特征。抗可提取核抗原（干燥综合征最特异的血清学标志物）抗体的阳性率为10.4%。我们的结论是，周围神经病变和孤立性干燥综合征形成一个独特的综合征，其中神经病变是目前的特点和干燥症是很容易被忽视;感觉多发性神经病变和多神经节病变占主导地位;血清学是确诊的，但非常不敏感;和腺体外疾病，包括血管炎，是不常见的典型干燥综合征相比。诊断时需要检查眼部或唾液受累情况，神经活检证实炎症是支持性的。这种疾病的定义的改进应允许进一步研究的自然史和免疫治疗的疗效。
Peripheral neuropathy occurs in Sjogren's syndrome, a disorder in which systemic immunologic phenomena, including vasculitis, are common. Neuropathy also occurs with isolated sicca complex (keratoconjunctivitis sicca and xerostomia); whether this represents a distinct syndrome is unclear. We retrospectively studied 54 patients with sicca complex and peripheral neuropathy to determine mode of presentation, neuropathic patterns, frequency and pattern of serologic abnormalities, and frequency of systemic disease, including necrotizing vasculitis. Peripheral neuropathy was the presenting problem in 87%. Although sicca symptoms occurred in 93%, they were a presenting complaint in only 11% and were usually mild, reported only after specific inquiry. Minor salivary gland biopsy was positive in 73%. Sensory neuropathies strongly predominated; 61% of patients manifested either sensory polyneuropathy or polyganglionopathy. Less common patterns included sensorimotor polyneuropathy (17%) and polyradiculoneuropathy (11%). Vasculitic neuropathy was demonstrated in only two patients, but nonspecific epineurial inflammation was present in 70% of nerve biopsies. Clinical evidence of systemic disease was uncommon, particularly in the sensory polyganglionopathy group, in whom extraglandular features other than weight loss occurred in only 1 of 12 patients. Antibodies to extractable nuclear antigens, the most specific serologic marker of Sjogren's syndrome, were present in 10.4%. We conclude that peripheral neuropathy and isolated sicca complex form a distinctive syndrome in which neuropathy is the presenting feature and sicca is easily overlooked; sensory polyneuropathy and polyganglionopathy predominate; serology is confirmatory but very insensitive; and extraglandular disease, including vasculitis, is uncommon compared with typical Sjogren's syndrome. Tests of ocular or salivary involvement are needed for diagnosis, and demonstration of inflammation in biopsied nerve is supportive. Improved definition of this disorder should permit further studies of natural history and efficacy of immunotherapy.