Autoinflammatory & rare systemic diseases
Longitudinal profiling reveals immune dynamics and distinct plasma cell signatures during B-cell depletion in IgG4-related disease
Authors' conclusion
NMU-expressing IGHV1-69+ plasma cells represent a pathogenic effector population driving IgG4-RD recurrence.
Study context
- Population
- Patients with igG4-related disease (IgG4-RD) undergoing RTX treatment; matched blood and tissue samples from submandibular gland and lymph nodes119 peripheral blood samples
- Comparison
- IgG4-RD during RTX-induced B-cell depletion, with SjD and healthy individuals as controls
- Outcome
- Longitudinal immune dynamics during B-cell depletion
Methodological detailslongitudinal, multiomics, single-cell transcriptomics, B-cell receptor (BCR) sequencing, serum proteomics
- Longitudinal
- Multiomics
- Single-cell transcriptomics
- BCR sequencing
- Serum proteomics
Main finding
As reported in the abstract
- Previously unrecognised, disease-specific IgG4+ plasma cell subset with aberrant expression of the neuropeptide NMU and highly skewed IGHV1-69 usage
- Circulating CD4+Tfh displayed signatures consistent with chronic antigen stimulation and type 2 immunity
- Markedly expanded dnT highly expressed IL10, suggesting involvement in immune regulation and isotype class switching
- After B-cell depletion: reconstitution dynamics of pathogenic plasma cells, with concurrent shifts in CD4+Tfh and dnT mirroring remission and relapse, and amelioration of proinflammatory immune profiles
Original publication
Longitudinal profiling reveals immune dynamics and distinct plasma cell signatures during B-cell depletion in IgG4-related disease. Ann Rheum Dis. Published 13 June 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →