PT  - JOURNAL ARTICLE
AU  - Su-Hyun Kim
AU  - Hyunmin Jang
AU  - Na Young Park
AU  - Yeseul Kim
AU  - So-Yeon Kim
AU  - Min Young Lee
AU  - Jae-Won Hyun
AU  - Ho Jin Kim
TI  - Discontinuation of Immunosuppressive Therapy in Patients With Neuromyelitis Optica Spectrum Disorder With Aquaporin-4 Antibodies
AID  - 10.1212/NXI.0000000000000947
DP  - 2021 Mar 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e947
VI  - 8
IP  - 2
4099  - http://nn.neurology.org/content/8/2/e947.short
4100  - http://nn.neurology.org/content/8/2/e947.full
SO  - Neurol Neuroimmunol Neuroinflamm2021 Mar 01; 8
AB  - 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.AQP4=aquaporin-4; AZA=azathioprine; DMT=disease-modifying therapies; EDSS=Expanded Disability Status Scale; IQR=interquartile range; IST=immunosuppressive therapy; MMF=mycophenolate mofetil; NMOSD=neuromyelitis optica spectrum disorder