{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T13:06:44Z","timestamp":1779196004477,"version":"3.51.4"},"reference-count":29,"publisher":"American Society for Clinical Investigation","issue":"15","license":[{"start":{"date-parts":[[2020,8,6]],"date-time":"2020-08-06T00:00:00Z","timestamp":1596672000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["P30AG053760"],"award-info":[{"award-number":["P30AG053760"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,8,6]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Our objective was to investigate whether primary Sj\u00f6gren\u2019s syndrome (pSS) is associated with multiple system atrophy (MSA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>We performed a retrospective cohort study assessing (a) rates of MSA in a cohort of patients with pSS and (b) rates of pSS in a cohort of patients with MSA. These data were compared with rates in respective control groups. We additionally reviewed the neuropathologic findings in 2 patients with pSS, cerebellar degeneration, parkinsonism, and autonomic dysfunction.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>\n                      Our cohort of 308 patients with pSS had a greater incidence of MSA compared with 4 large population-based studies and had a significantly higher prevalence of at least probable MSA (1% vs. 0%,\n                      <jats:italic>P<\/jats:italic>\n                      = 0.02) compared with 776 patients in a control cohort of patients with other autoimmune disorders. Our cohort of 26 autopsy-proven patients with MSA had a significantly higher prevalence of pSS compared with a cohort of 115 patients with other autopsy-proven neurodegenerative disorders (8% vs. 0%,\n                      <jats:italic>P<\/jats:italic>\n                      = 0.03). The 2 patients we described with pSS and progressive neurodegenerative disease showed classic MSA pathology at autopsy.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSION<\/jats:title>\n                    <jats:p>Our findings provide evidence for an association between MSA and pSS that is specific to both pSS, among autoimmune disorders, and MSA, among neurodegenerative disorders. The 2 cases we describe of autopsy-proven MSA support that MSA pathology can explain neurologic disease in a subset of patients with pSS. These findings together support the hypothesis that systemic autoimmune disease plays a role in neurodegeneration.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>FUNDING<\/jats:title>\n                    <jats:p>The Michigan Brain Bank is supported in part through NIH grant P30AG053760.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1172\/jci.insight.138619","type":"journal-article","created":{"date-parts":[[2020,7,9]],"date-time":"2020-07-09T12:01:47Z","timestamp":1594296107000},"source":"Crossref","is-referenced-by-count":5,"title":["Multiple system atrophy pathology is associated with primary Sj\u00f6gren\u2019s syndrome"],"prefix":"10.1172","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3888-5583","authenticated-orcid":true,"given":"Kyle S.","family":"Conway","sequence":"first","affiliation":[{"name":"Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, Michigan, USA."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9606-9402","authenticated-orcid":true,"given":"Sandra","family":"Camelo-Piragua","sequence":"additional","affiliation":[{"name":"Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, Michigan, USA."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amanda","family":"Fisher-Hubbard","sequence":"additional","affiliation":[{"name":"Department of Pathology, Homer Stryker M.D. School of Medicine, Western Michigan University, Kalamazoo, Michigan, USA."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William R.","family":"Perry","sequence":"additional","affiliation":[{"name":"Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, Michigan, USA."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2742-6469","authenticated-orcid":true,"given":"Vikram 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