Treatment of Neuromyelitis Optica Spectrum Disorders

被引:24
|
作者
Chan, Koon-Ho [1 ,2 ,3 ]
Lee, Chi-Yan [1 ,2 ]
机构
[1] Univ Hong Kong, LKS Fac Med, Dept Med, Hong Kong, Peoples R China
[2] Univ Hong Kong, LKS Fac Med, Neuroimmunol & Neuroinflammat Res Lab, Hong Kong, Peoples R China
[3] Univ Hong Kong, LKS Fac Med, Res Ctr Heart Brain Hormone & Hlth Aging, Hong Kong, Peoples R China
关键词
neuromyelitis optica spectrum disorders; aquaporin-4; autoimmunity; AQP4-IgG; B lymphocytes; T lymphocytes; immunosuppressive therapies; INTERLEUKIN-6 RECEPTOR BLOCKADE; REGULATORY B-CELLS; PLASMA-EXCHANGE; INTRAVENOUS IMMUNOGLOBULIN; OPEN-LABEL; TREATMENT OUTCOMES; CHINESE PATIENTS; CLINICAL-COURSE; ACUTE RELAPSES; DOUBLE-BLIND;
D O I
10.3390/ijms22168638
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune central nervous system (CNS) inflammatory disorder that can lead to serious disability and mortality. Females are predominantly affected, including those within the reproductive age. Most patients develop relapsing attacks of optic neuritis; longitudinally extensive transverse myelitis; and encephalitis, especially brainstem encephalitis. The majority of NMOSD patients are seropositive for IgG autoantibodies against the water channel protein aquaporin-4 (AQP4-IgG), reflecting underlying aquaporin-4 autoimmunity. Histological findings of the affected CNS tissues of patients from in-vitro and in-vivo studies support that AQP4-IgG is directly pathogenic in NMOSD. It is believed that the binding of AQP4-IgG to CNS aquaporin-4 (abundantly expressed at the endfoot processes of astrocytes) triggers astrocytopathy and neuroinflammation, resulting in acute attacks. These attacks of neuroinflammation can lead to pathologies, including aquaporin-4 loss, astrocytic activation, injury and loss, glutamate excitotoxicity, microglial activation, neuroinflammation, demyelination, and neuronal injury, via both complement-dependent and complement-independent pathophysiological mechanisms. With the increased understanding of these mechanisms underlying this serious autoimmune astrocytopathy, effective treatments for both active attacks and long-term immunosuppression to prevent relapses in NMOSD are increasingly available based on the evidence from retrospective observational data and prospective clinical trials. Knowledge on the indications and potential side effects of these medications are essential for a clear evaluation of the potential benefits and risks to NMOSD patients in a personalized manner. Special issues such as pregnancy and the coexistence of other autoimmune diseases require additional concern and meticulous care. Future directions include the identification of clinically useful biomarkers for the prediction of relapse and monitoring of the therapeutic response, as well as the development of effective medications with minimal side effects, especially opportunistic infections complicated by long-term immunosuppression.
引用
收藏
页数:27
相关论文
共 50 条
  • [1] Treatment of neuromyelitis optica/neuromyelitis optica spectrum disorders with methotrexate
    Ramanathan, Ramnath Santosh
    Malhotra, Konark
    Scott, Thomas
    [J]. BMC NEUROLOGY, 2014, 14
  • [2] Treatment of Neuromyelitis Optica/Neuromyelitis Optica Spectrum Disorders with Methotrexate
    Ramanathan, Ramanth Santosh
    Malhotra, Konark
    Scott, Thomas
    [J]. ANNALS OF NEUROLOGY, 2013, 74 : S22 - S22
  • [3] Treatment of neuromyelitis optica/neuromyelitis optica spectrum disorders with methotrexate
    Ramnath Santosh Ramanathan
    Konark Malhotra
    Thomas Scott
    [J]. BMC Neurology, 14
  • [4] Treatment of neuromyelitis optica spectrum disorders
    Romeo, Andrew R.
    Segal, Benjamin M.
    [J]. CURRENT OPINION IN RHEUMATOLOGY, 2019, 31 (03) : 250 - 255
  • [5] Neuromyelitis optica and neuromyelitis optica spectrum disorders
    Marignier, Romain
    Calvo, Alvaro Cobo
    Vukusic, Sandra
    [J]. CURRENT OPINION IN NEUROLOGY, 2017, 30 (03) : 208 - 215
  • [6] Early treatment in neuromyelitis optica spectrum disorders
    Radaelli, M.
    Sangalli, F.
    Guerrieri, S.
    Moiola, L.
    Esposito, F.
    Colombo, B.
    Romeo, M.
    Preziosa, P.
    Pensato, U.
    Greco, G.
    Comola, M.
    Comi, G.
    Martinelli, V.
    [J]. MULTIPLE SCLEROSIS JOURNAL, 2018, 24 : 130 - 130
  • [7] Treatment of relapses in neuromyelitis optica spectrum disorders
    Hradilek, P.
    [J]. CESKA A SLOVENSKA NEUROLOGIE A NEUROCHIRURGIE, 2020, 83 : S58 - S61
  • [8] Treatment of neuromyelitis optica spectrum disorders with azathioprine
    Lin, C. R.
    Tan, K.
    [J]. JOURNAL OF THE NEUROLOGICAL SCIENCES, 2009, 285 : S207 - S207
  • [9] Satralizumab for the Treatment of Neuromyelitis Optica Spectrum Disorders
    Gao, Yanli
    Zhang, Baoqi
    Yang, Junyi
    [J]. ANNALS OF PHARMACOTHERAPY, 2021, 55 (09) : 1167 - 1171
  • [10] Spectrum of Neuromyelitis optica spectrum disorders
    Khan, F. M.
    Dave, D.
    Rao, P.
    Nirhale, S.
    Rohatgi, S.
    Naphade, P.
    [J]. EUROPEAN JOURNAL OF NEUROLOGY, 2020, 27 : 685 - 685