Autoinflammatory & rare systemic diseases
CCR4+Tfh2 cells specifically produce IL-4 driving the pathological reaction in IgG4-related disease
Authors' conclusion
CCR4 serves as a marker for identifying Tfh2 cells that specifically produce IL-4. CCR4+Tfh2 cells are a widely present T cell population that infiltrates tertiary lymphoid tissues and storiform fibrosis of IgG4-RD.
Study context
- Population
- Treatment-naïve patients with active IgG4-related disease (IgG4-RD)IgG4-RD patients
- Participants
- 23 patients
- Comparison
- IgG4-RD vs healthy controls; CCR4+Tfh2 vs CCR4-Tfh2 cells
- IgG4-RDn = 23
- Outcome
- Whether CCR4 identifies IL-4-producing Tfh2 cells and its involvement in IgG4-RD
Methodological detailsmulti-colour flow cytometry, t-SNE, immunohistochemical staining
- Multi-colour flow cytometry
- t-SNE
- Immunohistochemical staining
Main finding
As reported in the abstract
- Tfh2 cells expressing CCR4 were identified as T follicular helper (Tfh) cells that specifically produce IL-4
- CCR4+Tfh2 cells showed higher GATA-3 and ICOS expression than CCR4-Tfh2 cells; no difference in BCL-6 and FOXP3
- Proportion of CCR4+Tfh2 cells in peripheral blood increased in IgG4-RD vs healthy controls; even more infiltrated affected lesions
- CCR4+GATA-3+Tfh2 cells diffusely infiltrated tertiary lymphoid tissues and storiform fibrosis lesions
- Proportion of CCR4+Tfh2 cells correlated significantly, specifically with serum IgG4 levels among clinical indicators
Further findings
Effect of GC therapy on CCR4+Tfh2 cells
- GC therapy did not correct the increased proportion of CCR4+Tfh2 cells
Original publication
CCR4+Tfh2 cells specifically produce IL-4 driving the pathological reaction in IgG4-related disease. Clin Exp Rheumatol. Published 28 May 2024. doi:10. 55563/ clinexprheumatol/ me9o6d
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