Autoinflammatory & rare systemic diseases

Treatment strategies for relapsing IgG4-related disease (IgG4-RD): 12-year experience from a prospective cohort

Rheumatology (Oxford)Published Prospective cohort study

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 0.0002
GC onlytendency to experience fewer relapses
0.51(0.26–1.03)
adjusted P 0.0823

Observational 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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