Briani Chiara, Doria Andrea, Ruggero Susanna, Toffanin Elisabetta, Luca Milena, Albergoni Maria Paola, D'Odorico Anna, Grassivaro Francesca, Lucchetta Marta, De Lazzari Franca, Balzani Italo, Battistin Leontino, Vernino Steven
Department of Neurosciences, University of Padova, Padova, Italy.
Autoimmunity. 2008 Feb;41(1):100-4. doi: 10.1080/08916930701619987.
About 2.5% of patients with idiopathic peripheral neuropathy or idiopathic dysautonomia have underlying celiac disease (CD). Antibodies to ganglioside have been reported in CD patients with neuropathy. No data are so far available on the presence in CD of acetylcholine receptor (AChR) antibodies. Muscle AChR antibodies are found in patients with myasthenia gravis, and ganglionic AChR antibodies in patients with autoimmune autonomic neuropathy.
To determine the frequency of AChR antibodies in CD patients and assess possible correlations with neurological manifestations.
Seventy CD patients (16 M, 54 F, mean age 36 years) underwent neurological and electrophysiological evaluation. AChR antibodies were detected with radioimmunoprecipitation assay. Sera from 15 age-matched patients with systemic lupus erythematosus (SLE) and 10 with Sjogren syndrome were studied as controls.
None of our CD patients complained of autonomic symptoms or fatigable weakness. Borderline titres (0.03-0.05 nmol/l) of ganglionic AChR antibodies were present in 4 patients, one affected with type I diabetes and one with subclinical neuropathy. Three of the 4 patients underwent cardiovascular autonomic function tests, which showed no abnormalities. Low levels of ganglionic AChR antibodies (0.05-0.10 nmol/l) were found in 2 SLE control patients, one of whom had a severe sicca complex. Muscle AChR antibodies (>1.0 nmol/l) were found in two CD patient and one control patient with SLE. Neither had symptoms or signs of myasthenia gravis.
CD is occasionally associated with neurologic disease, and with antibody reactivity to neuronal antigens. None of our CD patients had autonomic failure or significant levels of ganglionic AChR antibodies. Two CD patient and one control with SLE had muscle AChR antibodies without clinical evidence of myasthenia. The presence of antibodies in CD and in SLE patients may reflect a non-specific autoimmune response in these patients or may indicate subclinical autoimmune autonomic and neuromuscular involvement.
约2.5%的特发性周围神经病或特发性自主神经功能障碍患者存在潜在的乳糜泻(CD)。在患有神经病变的CD患者中已报告有神经节苷脂抗体。目前尚无关于CD患者中是否存在乙酰胆碱受体(AChR)抗体的数据。重症肌无力患者体内可检测到肌肉型AChR抗体,自身免疫性自主神经病变患者体内可检测到神经节型AChR抗体。
确定CD患者中AChR抗体的频率,并评估其与神经表现的可能相关性。
70例CD患者(男性16例,女性54例,平均年龄36岁)接受了神经学和电生理学评估。采用放射免疫沉淀法检测AChR抗体。研究了15例年龄匹配的系统性红斑狼疮(SLE)患者和10例干燥综合征患者的血清作为对照。
我们的CD患者均无自主神经症状或易疲劳性肌无力。4例患者存在临界水平(0.03 - 0.05 nmol/l)的神经节型AChR抗体,其中1例患有1型糖尿病,1例患有亚临床神经病变。这4例患者中的3例接受了心血管自主功能测试,结果均无异常。2例SLE对照患者存在低水平(0.05 - 0.10 nmol/l)的神经节型AChR抗体,其中1例患有严重的干燥综合征。2例CD患者和1例SLE对照患者存在肌肉型AChR抗体(>1.0 nmol/l)。二者均无重症肌无力的症状或体征。
CD偶尔与神经疾病相关,并与对神经元抗原的抗体反应性有关。我们的CD患者均无自主神经功能衰竭或显著水平的神经节型AChR抗体。2例CD患者和1例SLE对照患者存在肌肉型AChR抗体,但无重症肌无力的临床证据。CD患者和SLE患者体内抗体的存在可能反映了这些患者的非特异性自身免疫反应,或可能表明存在亚临床自身免疫性自主神经和神经肌肉受累。