BACKGROUND Patients with systemic sclerosis (SSc) complicated by gastrointestinal dysmotility are difficult to treat and have high mortality. To clarify the pathogenesis of gastrointestinal manifestations, we aimed to demonstrate the association among the clinical features of SSc, the serological markers, the autoantibodies against nicotinic acetylcholine receptor at autonomic ganglia (gAChR). METHODS Fifty patients were enrolled and divided into two groups according to the presence or absence of gastrointestinal manifestations, and the characteristics were analyzed between these two groups. We measured biomarkers and the autoantibodies against two gAChRα3 and β4 subunits to test sera samples. Furthermore, patients were classified based on the presence or absence of anti-gAChR autoantibodies, and their clinical features were compared. RESULTS In patients with SSc and gastrointestinal manifestations, digital ulcers were more frequent (p = 0.050) and VEGF expression was significantly higher (p = 0.038). Seven subjects with SSc were seropositive for α3 subunit, whereas one patient was seropositive for β4 subunit. The mean level of anti-gAChRα3 autoantibodies in SSc patients with gastrointestinal manifestations was significantly higher than that in SSc patients without gastrointestinal manifestations (p = 0.001). The group of patients with SSc and gAChR autoantibodies had significantly higher endostatin levels (p = 0.046). CONCLUSIONS This study is the first to demonstrate that clinical characteristics of SSc patients with seropositivity for gAChR autoantibodies. Patients with SSc have circulating autoantibodies against gAChR, which may contribute to gastrointestinal manifestations associated with this disease, suggesting that gAChR-mediated autonomic neurotransmission may provide a pathomechanism for gastrointestinal dysmotility in SSc.

中文翻译：

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使用抗 gAChR 抗体检测系统性硬化症患者的胃肠道表现。


背景系统性硬化症（SSc）并发胃肠动力障碍患者治疗困难且死亡率高。为了阐明胃肠道表现的发病机制，我们旨在证明 SSc 的临床特征、血清学标志物、自主神经节烟碱乙酰胆碱受体 (gAChR) 自身抗体之间的关联。方法 纳入50例患者，根据有无胃肠道表现分为两组，分析两组特点。我们测量了针对两个 gAChRα3 和 β4 亚基的生物标志物和自身抗体来测试血清样本。此外，根据是否存在抗 gAChR 自身抗体对患者进行分类，并比较他们的临床特征。结果 在患有 SSc 和胃肠道表现的患者中，指部溃疡更常见（p = 0.050），并且 VEGF 表达显着更高（p = 0.038）。 7 名 SSc 受试者的 α3 亚基血清呈阳性，而 1 名患者的 β4 亚基血清呈阳性。有胃肠道表现的 SSc 患者抗 gAChRα3 自身抗体的平均水平显着高于无胃肠道表现的 SSc 患者 (p = 0.001)。具有 SSc 和 gAChR 自身抗体的患者组的内皮抑素水平显着较高 (p = 0.046)。结论 本研究首次证明 gAChR 自身抗体血清阳性的 SSc 患者的临床特征。 SSc 患者体内存在针对 gAChR 的循环自身抗体，这可能导致与该疾病相关的胃肠道表现，表明 gAChR 介导的自主神经传递可能为 SSc 胃肠道运动障碍提供病理机制。