Anti-ganglionic AChR antibodies in Japanese patients with motility disorders

Anti-ganglionic AChR antibodies in Japanese patients with motility disorders
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DOI:
10.1007/s00535-018-1477-8
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发表时间:
2018-12-01
影响因子:
6.3
通讯作者:
Nakane, Shunya
Nakane, Shunya
中科院分区:
医学1区
文献类型:
--
作者:
Mukaino, Akihiro;Minami, Hitomi;Nakane, Shunya

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背景胃肠道动力障碍的自身免疫基础是多种自身抗体的存在。尚不清楚胃肠道动力障碍是自身免疫性自主神经节病(AAG)的特征还是与循环抗神经节乙酰胆碱受体(gAChR)抗体(Abs)相关。本研究的目的是确定自主神经功能障碍,抗gAChR抗体,和临床特征的患者胃肠道动力障碍,包括贲门失弛缓症和慢性假性肠梗阻(锡波)之间的关联。在2012年至2017年期间,从123名血清阳性AAG患者中获得了样本。二是前瞻性研究。分别在2014年至2016年以及2013年至2017年期间获得了28例贲门失弛缓症患者和14例锡波患者的样本。在第一项研究中,我们分析了血清阳性AAG患者的临床资料。在第二项研究中,我们比较了临床资料,自主症状,血清阳性,血清阴性贲门失弛缓症,和锡波groups.ResultsIn第一项研究中,我们确定了10例(8.1%)谁提出贲门失弛缓症,或胃轻瘫,或麻痹性肠梗阻之间的抗体筛查结果。在第二项研究中,我们在21.4%的贲门失弛缓症患者和50.0%的锡波患者中检测到抗gAChR抗体。虽然贲门失弛缓症和锡波患者表现出广泛的自主神经功能障碍,膀胱功能障碍观察到血清阳性患者与锡波作为一个突出的临床特征dysautonomia.ConclusionsThese结果表明一个显着的患病率贲门失弛缓症和锡波患者的抗gAChR抗体。抗gAChR抗体可能介导自主神经功能障碍,导致这些GI动力障碍的自身免疫机制。
BackgroundThe existence of several autoantibodies suggests an autoimmune basis for gastrointestinal (GI) dysmotility. Whether GI motility disorders are features of autoimmune autonomic ganglionopathy (AAG) or are related to circulating anti-ganglionic acetylcholine receptor (gAChR) antibodies (Abs) is not known. The aim of this study was to determine the associations between autonomic dysfunction, anti-gAChR Abs, and clinical features in patients with GI motility disorders including achalasia and chronic intestinal pseudo-obstruction (CIPO).MethodsFirst study: retrospective cohort study and laboratory investigation. Samples from 123 patients with seropositive AAG were obtained between 2012 and 2017. Second study: prospective study. Samples from 28 patients with achalasia and 14 patients with CIPO were obtained between 2014 and 2016, and 2013 and 2017, respectively. In the first study, we analyzed clinical profiles of seropositive AAG patients. In the second study, we compared clinical profiles, autonomic symptoms, and results of antibody screening between seropositive, seronegative achalasia, and CIPO groups.ResultsIn the first study, we identified 10 patients (8.1%) who presented with achalasia, or gastroparesis, or paralytic ileus. In the second study, we detected anti-gAChR Abs in 21.4% of the achalasia patients, and in 50.0% of the CIPO patients. Although patients with achalasia and CIPO demonstrated widespread autonomic dysfunction, bladder dysfunction was observed in the seropositive patients with CIPO as a prominent clinical characteristic of dysautonomia.ConclusionsThese results demonstrate a significant prevalence of anti-gAChR antibodies in patients with achalasia and CIPO. Anti-gAChR Abs might mediate autonomic dysfunction, contributing to autoimmune mechanisms underlying these GI motility disorders.