Autoinflammatory & rare systemic diseases
Intermediate-term follow-up and damage in IgG4-related disease: a single centre experience
Authors' conclusion
At diagnosis, damage was already present in nearly two-thirds of our patients. During follow-up, damage progressively accumulated and was associated with patient death.
Study context
- Population
- Patients with IgG4-related disease (IgG4-RD) diagnosed and monitored at a rheumatology centre, January 2012–December 202455 followed
- Participants
- 59 patients
- Outcome
- Damage progression (CIC IgG4-RD Damage Index) at baseline, 12 months, 24 months and last follow-up visit
Methodological detailssingle-centre, Cox proportional-hazards regression
- Single-centre
- Cox proportional-hazards regression
Main finding
As reported in the abstract
Damage (CIC IgG4-RD DI)
Damage at diagnosis
36
65.5%
Damage at 12-month visit
37 of 50
74%
Damage at 24-month visit (subgroup followed >24 months)
29 of 37
78.4%
Damage at last follow-up visit (subgroup followed >24 months)
32 of 37
86.5%
Observational data: associations, not effects.
Further findings
Damage at last visit as risk factor for death
| HR (95% CI) | P value | ||
|---|---|---|---|
| Damage at last visit | 1.5 (1.0–2.2) | P = 0.036 | |
| 0.512Hazard ratio (log scale) |
| Outcome | HR (95% CI) | P value |
|---|---|---|
| Relapsing disease course as factor associated with damage at last visit | 5.3 (1.1–24,4) | P = 0.035 |
Death during follow-up
- Deaths
- 8 (14.5%)
- Deaths related to IgG4-RD or its treatment
- 4
Original publication
Intermediate-term follow-up and damage in IgG4-related disease: a single centre experience. Clin Exp Rheumatol. Published 10 November 2025. doi:10.
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