Autoinflammatory & rare systemic diseases
First‐Year IgG Dynamics in IgG4 ‐Related Disease: A Critical Window for Predicting Long‐Term Outcomes
Main finding · Relapse by 12-month IgG normalizationResult — go to the resultHazard ratio 2.67 95% CI 1.43–4.99Authors' conclusion
Baseline IgG elevation marks more severe IgG4-RD phenotypes. The first treatment year achieving IgG normalization represents a critical prognostic biomarker. Failure to normalize IgG within 12 months markedly increases relapse risk.
Study context
- Population
- Newly treated patients with IgG4-related disease (IgG4-RD)
- Participants
- 274 participants
- Exposure
- Elevated vs normal baseline serum IgG; 12-month IgG normalization vs failure
- Elevated IgGn = 186
- Normal IgGn = 88reference
- Outcome
- Prognostic value of longitudinal serum IgG dynamics for relapse
- Follow-up
- 12 months
Methodological detailscovariance analysis adjusted for baseline IgG, Kaplan-Meier curves and Cox regression
- Covariance analysis adjusted for baseline IgG
- Kaplan-Meier curves and Cox regression
Main finding
Relapse by 12-month IgG normalization
Hazard ratio
2.67
95% CI 1.43–4.99
P = 0.002
By group
Failure of 12-month IgG normalization
HR 2.67 (95% CI 1.43–4.99); P = 0.002
As reported in the abstract
Observational data: associations, not effects.
Further findings
| Outcome | HR | P value |
|---|---|---|
| Relapse-free survival, meanIgG normalization vs No IgG normalization | log-rank P < 0.001 | |
| Relapse by treatment intensity (multivariate Cox)GCs + weak IMs vs GC-sparing | 0.36 | P = 0.012 |
| Relapse by treatment intensity (multivariate Cox)GCs + potent IMs vs GC-sparing | 0.40 | P = 0.020 |
- IgG normalization after treatment in elevated IgG
- 79.6%
- Relapse-free survival, mean: IgG normalization mean 33.20 (95% CI 32.21–34.19) months
- Relapse-free survival, mean: No IgG normalization mean 27.71 (95% CI 24.29–31.13) months
- IgG normalization by regimen: Glucocorticoid (GC)-based 85.2%
- IgG normalization by regimen: GC-sparing 66.7%
IgG normalization after treatment in elevated IgG
- Greatest IgG decline in the first year
Baseline phenotype with elevated IgG
- Elevated baseline IgG associated with more severe phenotypes: male predominance, older age, higher responder index scores, more internal organ involvement, hypocomplementemia, elevated ESR/CRP; IgG1 and IgG4 mainly contributed
Original publication
First‐Year IgG Dynamics in IgG4 ‐Related Disease: A Critical Window for Predicting Long‐Term Outcomes. Arthritis Rheumatol. Published 23 March 2026. doi:10.
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