{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T10:15:01Z","timestamp":1778840101389,"version":"3.51.4"},"reference-count":38,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T00:00:00Z","timestamp":1778803200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T00:00:00Z","timestamp":1778803200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Autoimmune autonomic ganglionopathy (AAG) is a rare but potentially treatable cause of severe autonomic failure. Evidence guiding long\u2010term immunotherapy, treatment sequencing, and residual autonomic impairment is limited. We evaluated long\u2010term treatment response, residual autonomic dysfunction, and relapse patterns in patients with seropositive AAG.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with seropositive AAG undergoing longitudinal follow\u2010up with repeated quantitative autonomic assessments before and after immunotherapy were included. Residual autonomic impairment was assessed using cardiovascular, pupillary, and urinary autonomic biomarkers, need for anti\u2010hypotensive medications or catheterisation, and patient\u2010reported symptoms measured by the COMPASS\u201031 questionnaire.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of 18 patients with seropositive AAG, 16 had longitudinal quantitative autonomic testing, antibody titres, COMPASS\u201031 scores, and documented medication and catheter use before and after immunotherapy. At baseline, all 16 had widespread autonomic failure. All received plasma exchange, 10 received intravenous immunoglobulins, and 11 were treated with oral prednisolone followed by steroid\u2010sparing agents. Treatment was associated with significant improvements in cardiovascular autonomic markers, antibody titres, and pupillary light responses, with non\u2010significant improvements in COMPASS\u201031 scores. Four patients discontinued catheterisation. Two patients were refractory to plasma exchange and intravenous immunoglobulins but responded to prednisolone. Two patients relapsed while receiving steroid\u2010sparing immunotherapy. High antibody titres correlated with the severity of cardiovascular and urinary dysfunction at disease onset, but not during follow\u2010up.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Prolonged stepwise immunosuppression in seropositive AAG is associated with sustained objective autonomic improvement, although residual impairment and relapse remain common. These findings support individualised, biomarker\u2010guided immunotherapy and highlight the need for ongoing supportive management of autonomic complications.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acn3.70421","type":"journal-article","created":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T10:03:35Z","timestamp":1778839415000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Long\u2010Term Efficacy of Immunotherapy in Autoimmune Autonomic Ganglionopathy\u2014A 10\u2010Year Follow Up Study"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4617-2559","authenticated-orcid":false,"given":"Giacomo","family":"Chiaro","sequence":"first","affiliation":[{"name":"Autonomic Unit National Hospital Neurology and Neurosurgery  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0860-9211","authenticated-orcid":false,"given":"Shiwen","family":"Koay","sequence":"additional","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases UCL Queen Square Institute of Neurology  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gordon T.","family":"Ingle","sequence":"additional","affiliation":[{"name":"Autonomic Unit National Hospital Neurology and Neurosurgery  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patricia","family":"McNamara","sequence":"additional","affiliation":[{"name":"Autonomic Unit National Hospital Neurology and Neurosurgery  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Watson","sequence":"additional","affiliation":[{"name":"Autonomic Unit National Hospital Neurology and Neurosurgery  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fion","family":"Bremner","sequence":"additional","affiliation":[{"name":"Department of Neuro\u2010Ophthalmology National Hospital for Neurology and Neurosurgery  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valeria","family":"Iodice","sequence":"additional","affiliation":[{"name":"Autonomic Unit National Hospital Neurology and Neurosurgery  London UK"},{"name":"Department of Translational Neuroscience and Stroke UCL Queen Square Institute of Neurology  London UK"},{"name":"NIHR University College London Hospitals Biomedical Research Centre  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,5,15]]},"reference":[{"key":"e_1_2_10_2_1","first-page":"355","article-title":"Pure Pan\u2010Dysautonomia With Recovery","volume":"94","author":"Young R. 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