Autoinflammatory & rare systemic diseases
Expansion and surface makers of age-associated B cells in IgG4-related disease
Authors' conclusion
ABCs were expanded both in the peripheral blood and affected tissues of patients with IgG4-RD as well as in the lungs of LAT mice, indicating the potential roles of ABCs in IgG4-RD pathogenesis.
Study context
- Population
- Patients with IgG4-related disease (IgG4-RD), patients with other autoimmune diseases and healthy controls; LatY136F knock-in (LAT) IgG4-RD mouse model
- Participants
- 51 / 18 / 34 / 61
- Comparison
- Untreated IgG4-RD vs IgG4-RD in remission vs other autoimmune diseases vs age- and sex-matched healthy controls (HCs)
- Untreated IgG4-RDn = 51
- IgG4-RD in remissionn = 18
- Other autoimmune diseasesn = 34
- HCsn = 61reference
- Outcome
- Expression of ABCs (CD21-T-bet+CD11c+ in CD19+ B cells) and their surface markers
Methodological detailsflow cytometry, immunofluorescence, LatY136F knock-in mouse model
- Flow cytometry
- Immunofluorescence
- LatY136F knock-in mouse model
Main finding
As reported in the abstract
- Percentages and absolute numbers of ABCs in CD19+ B cells raised remarkably in untreated IgG4-RD vs HC, and reduced significantly after treatment
- Percentage of CD27+ABCs, DN2 B cells and activated naive B cells higher in IgG4-RD than in HCs and multiple autoimmune diseases; comparable with SLE
- CD19+CD21-CD11c+ B cells of IgG4-RD vs HC: upregulated CD86, TACI, CD38; downregulated CXCR3
- In IgG4-RD, CD19+CD21-CD11c+ vs CD19+CD21-CD11c- B cells: higher CD80, CXCR3, TACI, CD95, BAFF-R; lower CD86, CD27, CD38, CXCR5
- ABCs (CD11c+T-bet+ in B220+ cells) increased significantly in lungs of LAT vs wild type (WT) mice
Original publication
Expansion and surface makers of age-associated B cells in IgG4-related disease. Clin Exp Rheumatol. Published 2 February 2023. doi:10.
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