Autoinflammatory & rare systemic diseases
Extrafollicular Tph2-like cell dominant tissue pathotype is associated with relapse in IgG4-related disease
Authors' conclusion
Higher Tph2 cells and lower CD19+ B cells in blood and T-B-pathotype in lacrimal glands are potentially associated with increased relapse risk in IgG4-RD.
Study context
- Population
- Consecutive, active patients with IgG4-related disease (IgG4-RD) at initiation of remission induction therapymedian follow-up 150 weeks
- Participants
- 74 participants
- Exposure
- Relapse vs non-relapse
- Relapsen = 19
- Outcome
- Relapse after remission induction therapy
Methodological detailsconsecutive patients, independent validation cohort, automated image-based quantification of lacrimal gland biopsies
- Consecutive patients
- Independent validation cohort
- Automated image-based quantification of lacrimal gland biopsies
Main finding
As reported in the abstract
Baseline blood immune subsets
| Group | Value | CD19+ B cells (median, cells/µl) |
|---|---|---|
| Relapse | median 1.8 cells/μl | 82 |
| Non-relapse | median 0.9 cells/μl | 133 |
Value
Relapsemedian 1.8 cells/μl
Non-relapsemedian 0.9 cells/μl
CD19+ B cells (median, cells/µl)
Relapse82
Non-relapse133
- Relapse vs Non-relapse: P = 0.018 — higher in relapse
- Relapse vs Non-relapse: P = 0.002 — lower in relapse
- Relapse rate
- 19 patients (25.7%)
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Lacrimal gland pathotype and relapseT-B-pathotype vs B-pathotype · higher relapse rate with T-B-pathotype | P = 0.022 |
Lacrimal gland pathotype and relapse
- CX3CR1+ Tph2-like cells accumulated in extrafollicular regions of lacrimal glands with relatively low B cell infiltration (T-B-pathotype)
- Findings reproduced in an independent validation set
Original publication
Extrafollicular Tph2-like cell dominant tissue pathotype is associated with relapse in IgG4-related disease. Rheumatology (Oxford). Published 12 August 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →