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. 2021 Feb 23;8(2):e947.
doi: 10.1212/NXI.0000000000000947. Print 2021 Mar.

Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies

Su-Hyun Kim  1 , Hyunmin Jang  2 , Na Young Park  2 , Yeseul Kim  1 , So-Yeon Kim  1 , Min Young Lee  2 , Jae-Won Hyun  2 , Ho Jin Kim  1
Affiliations

Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies

Su-Hyun Kim et al. Neurol Neuroimmunol Neuroinflamm. .

Erratum in

Abstract

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.

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Figures

Figure
Figure. Temporal Association of Clinical Relapse With Discontinuation of Immunosuppressive Therapy and AQP4 Antibody Status in 6 Patients With Neuromyelitis Optica Spectrum Disorder
N, AQP4 antibody negative, 1–4+ semiquantitative AQP4 antibody positive score. AQP4 = aquaporin-4; AZA = azathioprine; MMF = mycophenolate mofetil.
See this image and copyright information in PMC

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