Aberrant bone marrow granulopoiesis generates neutrophils that promote B cell activity in neuromyelitis optica spectrum disorder - PubMed
2 days ago
- #autoimmunity
- #neutrophils
- #B cells
- Bone marrow hematopoietic stem and progenitor cells (HSPCs) play a role in systemic immunity.
- Neuromyelitis optica spectrum disorder (NMOSD) is a B cell-mediated autoimmune disease with autoantibodies against aquaporin-4 (AQP4-IgG).
- Aberrant bone marrow granulopoiesis in NMOSD leads to increased ISG15+ neutrophils producing B cell-activating factor (BAFF).
- BAFF-producing neutrophils drive maturation of antibody-secreting cells and autoantibody production.
- Hyperactivated JAK-STAT signaling mediates aberrant granulopoiesis in NMOSD.
- B cell depletion therapy relapse cases still show aberrant granulopoiesis.
- Belimumab, a BAFF-targeting monoclonal antibody, reduces autoantibody titers and relapses.
- Targeting the bone marrow niche could be a treatment strategy for NMOSD and other B cell-mediated autoimmune diseases.