Background <p>Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease characterized by inflammatory demyelinating lesions affecting the optic nerve and spinal cord. As a rare condition, its clinical management—particularly in special populations such as pregnant patients—remains challenging. Inebilizumab, a B-cell-depleting biological agent used in aquaporin-4 immunoglobulin G-positive(AQP4-IgG+) NMOSD treatment, has demonstrated efficacy in disease control. However, no prior clinical reports have described its use in pregnant patients or the associated long-term maternal, fetal, and disease outcomes, leaving a critical gap in evidence. This case report, therefore, aims to address this knowledge deficit by presenting the first detailed account of an unexpected pregnancy in a patient receiving inebilizumab maintenance therapy, alongside the subsequent management strategies and outcomes.</p> Case presentation <p>We report a female patient with AQP4-IgG + NMOSD who experienced an unexpected pregnancy during three cycles of inebilizumab maintenance therapy. A multidisciplinary team (neurologists, obstetricians, and immunologists) collaborated to develop an individualized treatment plan. Following confirmation of pregnancy, her therapy was adjusted to low-dose methylprednisolone combined with azathioprine. During pregnancy, monitoring revealed persistently low B-cell counts, with low serum AQP4-IgG titers in the second trimester. The patient achieved a safe delivery and promptly resumed inebilizumab post-partum. Notably, no NMOSD recurrence was observed during the pre-conception, pregnancy, or post-partum periods.</p> Conclusions <p>This study is the first to report the successful management of an unplanned pregnancy in AQP4-IgG + NMOSD patient receiving inebilizumab. It confirms the efficacy and safety of preconception targeted B-cell depletion, individualized immunotherapeutic maintenance under multidisciplinary guidance during pregnancy, and timely resumption of targeted therapy postpartum, providing important clinical references for optimizing perinatal treatment strategies in reproductive-aged AQP4-IgG + NMOSD patients.</p>

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Unexpected pregnancy in a patient with AQP4-IgG + NMOSD after treatment with inebilizumab: a case report

  • Feiyan Wan,
  • Xia Wu,
  • Yuanyuan Tan,
  • Yuefei Guo,
  • Yuge Wang,
  • Wei Qiu,
  • Yaqing Shu,
  • Li Xiao

摘要

Background

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease characterized by inflammatory demyelinating lesions affecting the optic nerve and spinal cord. As a rare condition, its clinical management—particularly in special populations such as pregnant patients—remains challenging. Inebilizumab, a B-cell-depleting biological agent used in aquaporin-4 immunoglobulin G-positive(AQP4-IgG+) NMOSD treatment, has demonstrated efficacy in disease control. However, no prior clinical reports have described its use in pregnant patients or the associated long-term maternal, fetal, and disease outcomes, leaving a critical gap in evidence. This case report, therefore, aims to address this knowledge deficit by presenting the first detailed account of an unexpected pregnancy in a patient receiving inebilizumab maintenance therapy, alongside the subsequent management strategies and outcomes.

Case presentation

We report a female patient with AQP4-IgG + NMOSD who experienced an unexpected pregnancy during three cycles of inebilizumab maintenance therapy. A multidisciplinary team (neurologists, obstetricians, and immunologists) collaborated to develop an individualized treatment plan. Following confirmation of pregnancy, her therapy was adjusted to low-dose methylprednisolone combined with azathioprine. During pregnancy, monitoring revealed persistently low B-cell counts, with low serum AQP4-IgG titers in the second trimester. The patient achieved a safe delivery and promptly resumed inebilizumab post-partum. Notably, no NMOSD recurrence was observed during the pre-conception, pregnancy, or post-partum periods.

Conclusions

This study is the first to report the successful management of an unplanned pregnancy in AQP4-IgG + NMOSD patient receiving inebilizumab. It confirms the efficacy and safety of preconception targeted B-cell depletion, individualized immunotherapeutic maintenance under multidisciplinary guidance during pregnancy, and timely resumption of targeted therapy postpartum, providing important clinical references for optimizing perinatal treatment strategies in reproductive-aged AQP4-IgG + NMOSD patients.