title: Insights from the ganglionic acetylcholine receptor autoantibodies in patients with Sjögren's syndrome.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  (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.
  
  
  RESULTS
  (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.
  
  
  CONCLUSIONS
  The present results suggest the possibility of anti-gAChR antibodies aiding the diagnostics of SS with autonomic dysfunction.
authors.affiliation: a Department of Clinical Neuroscience and Neurology , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan .
authors.forename: Akihiro
authors.initials: A
authors.lastname: Mukaino
authors.affiliation: b Department of Clinical Research and.|c Department of Neurology , Nagasaki Kawatana Medical Center , Nagasaki , Japan .
authors.forename: Shunya
authors.initials: S
authors.lastname: Nakane
authors.affiliation: b Department of Clinical Research and.
authors.forename: Osamu
authors.initials: O
authors.lastname: Higuchi
authors.affiliation: d Department of Immunology and Rheumatology , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan .
authors.forename: Hideki
authors.initials: H
authors.lastname: Nakamura
authors.affiliation: e Department of Cardiovascular Medicine, Nephrology and Neurology , University of the Ryukyu School of Medicine , Okinawa , Japan .
authors.forename: Tomo
authors.initials: T
authors.lastname: Miyagi
authors.affiliation: e Department of Cardiovascular Medicine, Nephrology and Neurology , University of the Ryukyu School of Medicine , Okinawa , Japan .
authors.forename: Kanako
authors.initials: K
authors.lastname: Shiroma
authors.affiliation: e Department of Cardiovascular Medicine, Nephrology and Neurology , University of the Ryukyu School of Medicine , Okinawa , Japan .
authors.forename: Takashi
authors.initials: T
authors.lastname: Tokashiki
authors.affiliation: f Department of Neurology , Kitano Hospital Medical Research Institute , Osaka , Japan .
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Fuseya
authors.affiliation: g Department of Neurology , Hiroshima University Hospital , Hiroshima , Japan .
authors.forename: Kazuhide
authors.initials: K
authors.lastname: Ochi
authors.affiliation: d Department of Immunology and Rheumatology , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan .
authors.forename: Masataka
authors.initials: M
authors.lastname: Umeda
authors.affiliation: h Department of Neurology , Sapporo Yamanoue Hospital , Sapporo , Japan .
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Nakazato
authors.affiliation: i Department of Pediatrics, Graduate School of Medical Science , Kyoto Prefectural University of Medicine , Kyoto , Japan .
authors.forename: Shinji
authors.initials: S
authors.lastname: Akioka
authors.affiliation: j Department of Neurology and Neurological Science, and Predictive and Preventive Medicine , Tokyo Medical and Dental University , Tokyo , Japan .
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Maruoka
authors.affiliation: k Department of Pediatrics , Uwajima City Hospital , Ehime , Japan .
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Hayashi
authors.affiliation: l Department of Neurology , Niigata City General Hospital , Niigata , Japan , and.
authors.forename: Shu-Ichi
authors.initials: S
authors.lastname: Igarashi
authors.affiliation: m Department of Neurology , Anjo Kosei Hospital , Aichi , Japan.
authors.forename: Katsunori
authors.initials: K
authors.lastname: Yokoi
authors.affiliation: c Department of Neurology , Nagasaki Kawatana Medical Center , Nagasaki , Japan .
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Maeda
authors.affiliation: c Department of Neurology , Nagasaki Kawatana Medical Center , Nagasaki , Japan .
authors.forename: Waka
authors.initials: W
authors.lastname: Sakai
authors.affiliation: c Department of Neurology , Nagasaki Kawatana Medical Center , Nagasaki , Japan .
authors.forename: Hidenori
authors.initials: H
authors.lastname: Matsuo
authors.affiliation: d Department of Immunology and Rheumatology , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan .
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kawakami
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011950:Receptors, Cholinergic / Q000276:immunology*; D012859:Sjogren's Syndrome / Q000097:blood / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D011950:Receptors, Cholinergic
keywords: Antiganglionic acetylcholine receptor antibodies; Autonomic dysfunction; Luciferase immunoprecipitation systems; Sjögren’s syndrome