Autoinflammatory & rare systemic diseases
Treatment strategies for relapsing IgG4-related disease (IgG4-RD): 12-year experience from a prospective cohort
Authors' conclusion
When treating relapse in IgG4-RD, GC-based treatment was associated with a lower hazard of recurrent relapse compared with only adjusting IMs, especially for those with internal organ involvement.
Study context
- Population
- Patients with IgG4-RD from a prospective cohort who experienced their first relapse and were followed for over 12 months (61.7% male; median age 54.0 years)
- Participants
- 259 patients
- Exposure
- Intensifying both glucocorticoids (GC) and immunosuppressants (IM), or GC only, vs IM only, at first relapse of IgG4-RD
- Outcome
- Recurrent relapses
Methodological detailsdecision tree model
- Decision tree model
Main finding
As reported in the abstract
Each group vs IM onlyHazard ratio (95% CI)P value
GC and IM
0.29(0.16–0.52)
adjusted P 0.0002GC onlytendency to experience fewer relapses
0.51(0.26–1.03)
adjusted P 0.0823Observational data: associations, not effects.
Further findings
Recurrent relapses in subgroup with internal organ involvement
- Differences more pronounced in the subgroup with internal organ involvement
Decision tree prediction of freedom from recurrent relapse (RI decrease to ≤2 and IgG4 decrease ≥25% after treatment)
- AUC in the testing set
- 0.728
Original publication
Treatment strategies for relapsing IgG4-RD: 12-year experience from a prospective cohort. Rheumatology (Oxford). Published 11 August 2026. doi:10. 1093/ rheumatology/ keag401
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