Vasculitis & polymyalgia rheumatica
Defects in B-lymphopoiesis and B-cell maturation underlie prolonged B-cell depletion in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis
Authors' conclusion
Prolonged depletion of B cells in patients with AAV after RTX therapy indicates a B-cell defect that is unmasked by RTX treatment. Our data indicate that impaired bone marrow B-lymphopoiesis results in a delayed recovery of peripheral B cells that may be further aggravated by a survival defect of B cells.
Study context
- Population
- Patients with AAV, treatment-naïve and rituximab (RTX)-treated
- Participants
- 91 patients
Methodological detailsspectral flow and mass cytometry, in vitro modelling
- Spectral flow and mass cytometry
- In vitro modelling
Main finding
As reported in the abstract
- Low transitional B-cell numbers in treatment-naïve AAV, suggesting impaired B-lymphopoiesis
- Reduced B-lymphoid precursors in bone marrow of treatment-naïve and RTX-treated AAV
- In vitro B-lymphopoiesis from AAV haematopoietic stem cells: intact, but slower and reduced immature B-cell development
- In a subgroup after RTX, transitional B cells did not translate into replenishment of naïve B cells, suggesting impaired peripheral B-cell maturation
- Low BAFF-receptor expression on B cells of RTX-treated AAV, with reduced survival in response to BAFF in vitro
Original publication
Defects in B-lymphopoiesis and B-cell maturation underlie prolonged B-cell depletion in ANCA-associated vasculitis. Ann Rheum Dis. Published 21 October 2024. doi:10.
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