University of Rochester Medical Center, Department of Neurology, 601 Elmwood Avenue, Box 673, Rochester, NY, United States.
University of Rochester Medical Center, Department of Neurology, 601 Elmwood Avenue, Box 673, Rochester, NY, United States.
Mult Scler Relat Disord. 2020 Jan;37:101442. doi: 10.1016/j.msard.2019.101442. Epub 2019 Oct 13.
Aquaporin-4 IgG (AQ4-IgG)-neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory CNS disease that is predominantly characterized by severe relapses of optic neuritis and longitudinally extensive transverse myelitis (LETM). Women are disproportionately affected by AQ4-NMOSD, usually with disease onset occurring between the ages of 35-45. This has significant implications during pregnancy, as disease activity in NMOSD does not remit during gestation. The optimal treatment of NMOSD during pregnancy has not been established.
Case report.
A 35-year old woman, 10 weeks pregnant, presented with bilateral optic neuritis and intractable hiccups. Workup revealed seropositive aquaporin-4 IgG. She was treated with pulse intravenous methylprednisolone and plasma exchange. Because of high risk for future relapse, Rituximab 1000 mg was given at weeks 15 and 17 of pregnancy. She had no further relapses during pregnancy. She delivered her daughter at 39 weeks without complication.
This case demonstrated a favorable outcome in administering rituximab for NMOSD with disease onset during pregnancy. This description of therapy for disease onset during pregnancy is novel, and adds to the few existing case reports of administering rituximab during pregnancy.
水通道蛋白 4 免疫球蛋白 G(AQ4-IgG)-视神经脊髓炎谱系疾病(NMOSD)是一种主要以严重视神经炎和长节段横贯性脊髓炎(LETM)复发为特征的自身免疫性中枢神经系统疾病。女性受 AQ4-NMOSD 的影响不成比例,通常在 35-45 岁之间发病。这在怀孕期间有重大影响,因为 NMOSD 的疾病活动在妊娠期间不会缓解。怀孕期间 NMOSD 的最佳治疗方法尚未确定。
病例报告。
一名 35 岁女性,怀孕 10 周,出现双侧视神经炎和顽固性呃逆。检查发现血清阳性水通道蛋白 4 免疫球蛋白 G。她接受了脉冲静脉注射甲基强的松龙和血浆置换治疗。由于未来复发的风险较高,在妊娠第 15 周和第 17 周给予利妥昔单抗 1000mg。她在怀孕期间没有再次复发。她在没有并发症的情况下于 39 周分娩。
本病例表明在怀孕期间 NMOSD 发病时使用利妥昔单抗治疗具有良好的效果。这种治疗方法在怀孕期间 NMOSD 发病的描述是新颖的,并增加了少数在怀孕期间使用利妥昔单抗的现有病例报告。