title: Autoimmune autonomic ganglionopathy with Sjögren's syndrome: significance of ganglionic acetylcholine receptor antibody and therapeutic approach.
abstract: Autoimmune autonomic ganglionopathy (AAG) is a disorder defined by antibodies to the nicotinic acetylcholine receptor of the autonomic ganglia. We report two patients with chronically progressing dysautonomia with Sjögren's syndrome (SS). The first case showed elevated titer of ganglionic acetylcholine receptor (AChR) antibody and improved with oral intake of prednisolone. In contrast, the second case showed no elevation of ganglionic AChR antibody titer and had poor response to immunomodulatory therapy. These two cases indicate that chronic AAG may be treatable by immunomodulatory therapy, and have relevance to SS.
authors.affiliation: Department of Neurology, Kyoto University Hospital, Kyoto, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kondo
authors.forename: Haruhisa
authors.initials: H
authors.lastname: Inoue
authors.forename: Takashi
authors.initials: T
authors.lastname: Usui
authors.forename: Tsuneyo
authors.initials: T
authors.lastname: Mimori
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Tomimoto
authors.forename: Steven
authors.initials: S
authors.lastname: Vernino
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Takahashi
mesh_terms: D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D005725:Ganglia, Autonomic / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D054969:Primary Dysautonomias / Q000175:diagnosis / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D011681:Pupil Disorders / Q000209:etiology; D054970:Pure Autonomic Failure / Q000175:diagnosis; D011978:Receptors, Nicotinic / Q000276:immunology*; D012261:Ribonucleoproteins / Q000276:immunology; D012791:Shy-Drager Syndrome / Q000209:etiology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D014555:Urination Disorders / Q000209:etiology; D000097585:SS-B Antigen; D000099207:SS-A Antigen
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D001324:Autoantigens; D007166:Immunosuppressive Agents; D011239:Prednisolone; D011978:Receptors, Nicotinic; D012261:Ribonucleoproteins; D000097585:SS-B Antigen; D000099207:SS-A Antigen; C035356:SS-A antibodies; C035357:SS-B antibodies