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

Clin Exp RheumatolPublished Prospective cohort study

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 involvement2.11 (1.27–3.50)P = 0.004
Relapse frequency1.40 (1.04–1.89)P = 0.028

Risk factor for damage accrual at 10 years

OR (95% CI)P value
Baseline pancreatic involvement2.89 (1.02–8.16)P = 0.045

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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