{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T23:50:41Z","timestamp":1793922641470,"version":"4.8.0"},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,7,26]],"date-time":"2017-07-26T00:00:00Z","timestamp":1501027200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Intern Med"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Inhibitory antibodies towards enzyme replacement therapy (\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      ) are associated with disease progression and poor outcome in affected male patients with lysosomal disorders such as Fabry disease (\n                      <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n                      ). However, little is known about the impact of immunosuppressive therapy on\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition in these patients with\n                      <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In this retrospective study, we investigated the effect of long\u2010term immunosuppression on\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition in male patients with\n                      <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n                      (\n                      <jats:italic>n<\/jats:italic>\n                      = 26) receiving immunosuppressive therapy due to kidney (\n                      <jats:italic>n<\/jats:italic>\n                      = 24) or heart (\n                      <jats:italic>n<\/jats:italic>\n                      = 2) transplantation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      No\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      \u2010na\u00efve transplanted patient (\n                      <jats:italic>n<\/jats:italic>\n                      = 8) developed antibodies within follow\u2010up (80 \u00b172 months) after\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      initiation. Seven (26.9%) patients were tested\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition positive prior to transplantation. No\n                      <jats:italic>de novo<\/jats:italic>\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition was observed after transplantation (\n                      <jats:italic>n<\/jats:italic>\n                      = 18). In patients treated with high dosages of immunosuppressive medication such as prednisolone, tacrolimus and mycophenolate\u2010mofetil\/mycophenolate acid,\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition decreased after transplantation (\n                      <jats:italic>n<\/jats:italic>\n                      = 12;\n                      <jats:italic>P<\/jats:italic>\n                      = 0.0160). Tapering of immunosuppression (especially prednisolone) seemed to re\u2010increase\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition (\n                      <jats:italic>n<\/jats:italic>\n                      = 4, median [range]: 16.6 [6.9; 36.9] %;\n                      <jats:italic>P<\/jats:italic>\n                      = 0.0972) over time. One\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition\u2010positive patient required interventions with steroid therapy and increased doses of tacrolimus, which also lowered\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      We conclude that the immunosuppressive maintenance therapy after transplantations seems to be sufficient to prevent\n                      <jats:italic>de novo<\/jats:italic>\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition in\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      \u2010na\u00efve patients. Intensified high dosages of immunosuppressive drugs are associated with decreased antibody titres and decreased\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition in affected patients, but did not result in long\u2010term protection. Future studies are needed to establish\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition\u2010specific immunosuppressive protocols with long\u2010term modulating properties to warrant an improved disease course in\n                      <jats:styled-content style=\"fixed-case\">ERT<\/jats:styled-content>\n                      inhibition\u2010positive males.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/joim.12647","type":"journal-article","created":{"date-parts":[[2017,7,6]],"date-time":"2017-07-06T10:57:54Z","timestamp":1499338674000},"page":"241-253","source":"Crossref","is-referenced-by-count":42,"title":["Impact of immunosuppressive therapy on therapy\u2010neutralizing antibodies in transplanted patients with Fabry disease"],"prefix":"10.1111","volume":"282","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8409-2886","authenticated-orcid":false,"given":"M.","family":"Lenders","sequence":"first","affiliation":[{"name":"Internal Medicine D Department of Nephrology, Hypertension and Rheumatology University Hospital Muenster Muenster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Oder","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I Divisions of Cardiology and Nephrology Comprehensive Heart Failure Center (CHFC) Fabry Center for Interdisciplinary Therapy (FAZIT) University of Wuerzburg Wuerzburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Nowak","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine University Hospital of Zurich and University of Zurich Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Canaan\u2010K\u00fchl","sequence":"additional","affiliation":[{"name":"Department of Medicine Division of Nephrology Campus Virchow\u2010Klinikum University Hospital Charit\u00e9 Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Arash\u2010Kaps","sequence":"additional","affiliation":[{"name":"Villa Metabolica Department for Pediatric and Adolescent Medicine University Medical Center of the Johannes Gutenberg\u2010University Mainz Mainz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Drechsler","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I Divisions of Cardiology and Nephrology Comprehensive Heart Failure Center (CHFC) Fabry Center for Interdisciplinary Therapy (FAZIT) University of Wuerzburg Wuerzburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Schmitz","sequence":"additional","affiliation":[{"name":"Institute of Sports Medicine Molecular Genetics of Cardiovascular Disease University Hospital Muenster Muenster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Nordbeck","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine I Divisions of Cardiology and Nephrology Comprehensive Heart Failure Center (CHFC) Fabry Center for Interdisciplinary Therapy (FAZIT) University of Wuerzburg Wuerzburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. 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