Association between neurosarcoidosis with autonomic dysfunction and anti-ganglionic acetylcholine receptor antibodies.
Association between neurosarcoidosis with autonomic dysfunction and anti-ganglionic acetylcholine receptor antibodies.
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DOI：
10.1007/s00415-021-10551-4
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发表时间：
2021-11
影响因子：
6
通讯作者：
Nakane S
中科院分区：
文献类型：
--
作者：
Oishi M;Mukaino A;Kunii M;Saito A;Arita Y;Koike H;Higuchi O;Maeda Y;Abiru N;Yamaguchi N;Kawano H;Tsuiki E;Tanaka T;Matsuo H;Katsuno M;Tanaka F;Tsujino A;Nakane S
确定神经结节病的自主神经功能障碍是否与抗神经节乙酰胆碱受体（gAChR）抗体相关，该抗体在自身免疫性自主神经节病中检测到。我们回顾性地从2012年至2018年期间1，381名患者的1787份血清样本中提取了结节病病例。通过荧光素酶免疫沉淀法测定针对α3和β4亚基的抗gAChR抗体，以确认每个病例的临床特征。我们总结了神经结节病伴严重植物神经功能障碍的文献综述，以确定相关的临床特征和结局。我们提取了3例伴有严重自主神经功能障碍的神经结节病新病例，其中2例抗gAChR抗体阳性：病例1抗β4亚基抗体阳性，病例2抗α3和β4亚基抗体均阳性。我们回顾了15例神经结节病和严重的自主神经功能障碍患者的病例，包括本文介绍的三例。体位性低血压和体位不耐受是最常见的症状。在各种类型的神经病中，小纤维神经病（SFN）是最常见的，9例中有7例表现出明确的SFN。8例中6例有节后纤维受损，其中3例123 I-MIBG心肌显像异常。11例中，除1例血清学阳性（病例2）外，10例对免疫治疗有反应。gAChR抗体的存在可能构成神经结节病中出现自主神经功能障碍的机制之一。
To determine whether autonomic dysfunction in neurosarcoidosis is associated with anti-ganglionic acetylcholine receptor (gAChR) antibodies, which are detected in autoimmune autonomic ganglionopathy. We retrospectively extracted cases of sarcoidosis from 1787 serum samples of 1,381 patients between 2012 and 2018. Anti-gAChR antibodies against the α3 and β4 subunit were measured by luciferase immunoprecipitation to confirm the clinical features of each case. We summarized literature reviews of neurosarcoidosis with severe dysautonomia to identify relevant clinical features and outcomes. We extracted three new cases of neurosarcoidosis with severe dysautonomia, among which two were positive for anti-gAChR antibodies: Case 1 was positive for antibodies against the β4 subunit, and Case 2 was positive for antibodies against both the α3 and β4 subunits. We reviewed the cases of 15 patients with neurosarcoidosis and severe dysautonomia, including the three cases presented herein. Orthostatic hypotension and orthostatic intolerance were the most common symptoms. Among the various types of neuropathy, small fiber neuropathy (SFN) was the most prevalent, with seven of nine cases exhibiting definite SFN. Six of eight cases had impaired postganglionic fibers, of which the present three cases revealed abnormality of 123I-MIBG myocardial scintigraphy. Of the 11 cases, 10 were responsive to immunotherapy, except one seropositive case (Case 2). The presence of gAChR antibodies may constitute one of the mechanisms by which dysautonomia arises in neurosarcoidosis.
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影响因子：
158.5
作者：
FAGAN, EA;MOOREGILLON, JC;TURNERWARWICK, M
通讯作者：
TURNERWARWICK, M
影响因子：
7.3
作者：
Kinloch AJ;Kaiser Y;Wolfgeher D;Ai J;Eklund A;Clark MR;Grunewald J
通讯作者：
Grunewald J
影响因子：
3.7
作者：
Moher D;Shamseer L;Clarke M;Ghersi D;Liberati A;Petticrew M;Shekelle P;Stewart LA;PRISMA-P Group
通讯作者：
PRISMA-P Group
影响因子：
168.9
作者：
Hoitsma, E;Marziniak, M;Drent, M
通讯作者：
Drent, M
影响因子：
4.6
作者：
Kudryavtsev, I.;Serebriakova, M.;Shoenfeld, Y.
通讯作者：
Shoenfeld, Y.