Autoinflammatory & rare systemic diseases
Evaluating and predicting disease damage accumulation of immunoglobulin G4-related disease (IgG4-RD) over ten years: utility of the IgG4-related Disease Damage Index
Authors' conclusion
The damage caused by IgG4-RD accumulates over time.
Study context
- Population
- Patients diagnosed with IgG4-RD followed for over 5 years in a prospective cohort99 followed for 10 years
- Participants
- 334 patients
- Exposure
- Increased damage vs stable damage subgroups
- Outcome
- Cumulative disease-induced damage (Chinese IgG4-RD Consortium IgG4-RD Damage Index)
- Follow-up
- Baseline (6 months), 5 years and 10 years
Methodological detailslong-term follow-up
- Long-term follow-up
Main finding
As reported in the abstract
DI score · baseline to 10 years
- Damage Index (DI) score at baseline
- 0.89
- DI score at 10 years
- 1.29
- 'Pancreatic' domain damage
- 13.4%
- 'Liver/biliary tree' domain damage
- 7.2%
- 'Other' domain damage
- 28.9%
- Malignancy, increase within 5 years
- 0.3% to 5.1%
- Diabetes mellitus, increase within 5 years
- 3.6% to 14.4%
Observational data: associations, not effects.
Further findings
Risk factors for damage accrual at 5 years
| OR (95% CI) | P value | ||
|---|---|---|---|
| Baseline pancreatic involvement | 2.11 (1.27–3.50) | P = 0.004 | |
| Relapse frequency | 1.40 (1.04–1.89) | P = 0.028 | |
| 0.20.5125Odds ratio (log scale) |
Risk factor for damage accrual at 10 years
| OR (95% CI) | P value | ||
|---|---|---|---|
| Baseline pancreatic involvement | 2.89 (1.02–8.16) | P = 0.045 | |
| 0.10.31310Odds ratio (log scale) |
Original publication
Evaluating and predicting disease damage accumulation of IgG4-RD over ten years: utility of the IgG4-related Disease Damage Index. Clin Exp Rheumatol. Published 18 March 2025. doi:10. 55563/ clinexprheumatol/ tfiy6x
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