Mukaino Akihiro, Nakane Shunya, Higuchi Osamu, Nakamura Hideki, Miyagi Tomo, Shiroma Kanako, Tokashiki Takashi, Fuseya Yasuhiro, Ochi Kazuhide, Umeda Masataka, Nakazato Tetsuya, Akioka Shinji, Maruoka Hiroyuki, Hayashi Masatoshi, Igarashi Shu-Ichi, Yokoi Katsunori, Maeda Yasuhiro, Sakai Waka, Matsuo Hidenori, Kawakami Atsushi
a Department of Clinical Neuroscience and Neurology , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan .
b Department of Clinical Research and.
Mod Rheumatol. 2016 Sep;26(5):708-15. doi: 10.3109/14397595.2016.1147404. Epub 2016 Jun 2.
It is not known whether autonomic neuropathy is a feature of Sjögren's syndrome (SS) or whether it is related to circulating antiganglionic acetylcholine receptor (gAChR) antibodies. The goal of the present study was to investigate the autonomic dysfunction in patients with SS and the associations between autonomic dysfunction, anti-gAChR antibodies, and clinical features of SS.
(1) The first observational study tested for the presence of gAChR antibodies in the serum samples from 39 patients with SS (absent information regarding autonomic symptoms) and healthy volunteers. (2) In the second study, serological and clinical data from 10 Japanese patients diagnosed with SS were reviewed. These patients showed autonomic dysfunction, and luciferase immunoprecipitation systems (LIPS) test was conducted to detect anti-α3 and anti-β4 gAChR antibodies. (3) In the final analysis, we combined the data of seropositive SS patients with autonomic symptom from the first study with all of the patients from the second study, and analyzed the clinical features.
(1) The LIPS assay revealed that anti-gAChRα3 and anti-gAChRβ4 antibodies were detected in the sera from patients with SS (23.1%, 9/39). Five of nine SS patients had autonomic symptoms. (2) Anti-α3 and anti-β4 gAChR antibodies were also detected in 80.0% (8/10) of patients with SS with autonomic symptoms. Six of the ten patients were diagnosed as having SS after neurological symptoms developed. These seropositive patients had predominant and severe autonomic symptoms and were diagnosed with autonomic neuropathy. (3) Thirteen of fifteen SS patients with autonomic symptoms (86.7%) were seropositive for anti-gAChR antibodies, and we confirmed sicca complex, orthostatic hypotension, upper and lower gastrointestinal (GI) symptoms, and bladder dysfunction at high rates.
The present results suggest the possibility of anti-gAChR antibodies aiding the diagnostics of SS with autonomic dysfunction.
目前尚不清楚自主神经病变是否为干燥综合征（SS）的特征，也不清楚其是否与循环中的抗神经节乙酰胆碱受体（gAChR）抗体有关。本研究的目的是调查SS患者的自主神经功能障碍，以及自主神经功能障碍、抗gAChR抗体与SS临床特征之间的关联。
（1）第一项观察性研究检测了39例SS患者（无自主神经症状信息）和健康志愿者血清样本中gAChR抗体的存在情况。（2）在第二项研究中，回顾了10例诊断为SS的日本患者的血清学和临床数据。这些患者表现出自主神经功能障碍，并进行了荧光素酶免疫沉淀系统（LIPS）检测以检测抗α3和抗β4 gAChR抗体。（3）在最终分析中，我们将第一项研究中具有自主神经症状的血清阳性SS患者的数据与第二项研究中的所有患者的数据合并，并分析了临床特征。
（1）LIPS检测显示，SS患者血清中检测到抗gAChRα3和抗gAChRβ4抗体（23.1%，9/39）。9例SS患者中有5例有自主神经症状。（2）在有自主神经症状的SS患者中，80.0%（8/10）也检测到抗α3和抗β4 gAChR抗体。10例患者中有6例在出现神经症状后被诊断为SS。这些血清阳性患者有主要且严重的自主神经症状，并被诊断为自主神经病变。（3）15例有自主神经症状的SS患者中有13例（86.7%）抗gAChR抗体血清阳性，我们还高比例地确认了干燥综合征、直立性低血压、上、下胃肠道（GI）症状和膀胱功能障碍。
目前的结果表明，抗gAChR抗体有助于诊断伴有自主神经功能障碍的SS。
