Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies
Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology..
OBJECTIVE: To evaluate the outcomes of immunosuppressive therapy (IST) discontinuation in patients with neuromyelitis optica spectrum disorder (NMOSD) after a sustained remission period.
METHODS: We retrospectively reviewed the medical records of 17 patients with antiaquaporin-4 antibody-positive NMOSD who discontinued IST after a relapse-free period of ≥3 years.
RESULTS: IST was discontinued at a median age of 40 years (interquartile range [IQR], 32-51) after a median relapse-free period of 62 months (IQR, 52-73). Among the 17 enrolled patients, 14 (82%) relapsed at a median interval of 6 months (IQR, 4-34) after IST discontinuation, 3 (18%) of whom experienced severe attacks; notably, all 3 of these patients had a history of severe attack before IST. These 3 patients received steroids, followed by plasma exchange for acute treatment, but 2 exhibited poor recovery and significant disability worsening at 6 months after relapse.
CONCLUSIONS: IST discontinuation may increase the risk of relapse in seropositive patients with NMOSD even after 5 years of remission. Given the potentially devastating consequence of a single attack of NMOSD, caution is advised with IST discontinuation, particularly in patients with severe attack before IST.
| Errataetall: |
ErratumIn: Neurol Neuroimmunol Neuroinflamm. 2021 Apr 19;8(4):. - PMID 33875580 |
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| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:8 |
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| Enthalten in: |
Neurology(R) neuroimmunology & neuroinflammation - 8(2021), 2 vom: 23. März |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Kim, Su-Hyun [VerfasserIn] |
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| Links: |
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| Themen: |
Aquaporin 4 |
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| Anmerkungen: |
Date Completed 28.10.2021 Date Revised 04.12.2021 published: Electronic-Print ErratumIn: Neurol Neuroimmunol Neuroinflamm. 2021 Apr 19;8(4):. - PMID 33875580 Citation Status MEDLINE |
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| doi: |
10.1212/NXI.0000000000000947 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM321810112 |
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| 500 | |a Date Completed 28.10.2021 | ||
| 500 | |a Date Revised 04.12.2021 | ||
| 500 | |a published: Electronic-Print | ||
| 500 | |a ErratumIn: Neurol Neuroimmunol Neuroinflamm. 2021 Apr 19;8(4):. - PMID 33875580 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. | ||
| 520 | |a OBJECTIVE: To evaluate the outcomes of immunosuppressive therapy (IST) discontinuation in patients with neuromyelitis optica spectrum disorder (NMOSD) after a sustained remission period | ||
| 520 | |a METHODS: We retrospectively reviewed the medical records of 17 patients with antiaquaporin-4 antibody-positive NMOSD who discontinued IST after a relapse-free period of ≥3 years | ||
| 520 | |a RESULTS: IST was discontinued at a median age of 40 years (interquartile range [IQR], 32-51) after a median relapse-free period of 62 months (IQR, 52-73). Among the 17 enrolled patients, 14 (82%) relapsed at a median interval of 6 months (IQR, 4-34) after IST discontinuation, 3 (18%) of whom experienced severe attacks; notably, all 3 of these patients had a history of severe attack before IST. These 3 patients received steroids, followed by plasma exchange for acute treatment, but 2 exhibited poor recovery and significant disability worsening at 6 months after relapse | ||
| 520 | |a CONCLUSIONS: IST discontinuation may increase the risk of relapse in seropositive patients with NMOSD even after 5 years of remission. Given the potentially devastating consequence of a single attack of NMOSD, caution is advised with IST discontinuation, particularly in patients with severe attack before IST | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
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| 650 | 7 | |a Autoantibodies |2 NLM | |
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| 700 | 1 | |a Kim, Yeseul |e verfasserin |4 aut | |
| 700 | 1 | |a Kim, So-Yeon |e verfasserin |4 aut | |
| 700 | 1 | |a Lee, Min Young |e verfasserin |4 aut | |
| 700 | 1 | |a Hyun, Jae-Won |e verfasserin |4 aut | |
| 700 | 1 | |a Kim, Ho Jin |e verfasserin |4 aut | |
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