Autoinflammatory & rare systemic diseases
Cumulative defined daily doses enable objective assessment of treatment intensity and outcome prediction in immunoglobulin G4-related disease and its subtypes
Authors' conclusion
Distinct proliferative and fibrotic phenotypes exhibit divergent risks and treatment responses.
Study context
- Population
- Patients fulfilling the 2020 revised comprehensive diagnostic criteria for Immunoglobulin G4-related disease (IgG4-RD)
- Participants
- 85 patients
- Exposure
- Proliferative vs fibrotic IgG4-RD subtypes; cumulative defined daily dose (DDD) of GCs and immunosuppressants
- Outcome
- Predictors of relapse and mortality
Methodological detailsmultivariate Cox regression, subgroup analysis by proliferative or fibrotic subtype
- Multivariate Cox regression
- Subgroup analysis by proliferative or fibrotic subtype
Main finding
As reported in the abstract
- Elevated ALT, higher serum IgG and greater steroid exposure (>1225 cDDD) independently predicted relapse
- Anaemia (Hb <10.9 g/dl) predicted both relapse and mortality
- Lower IgG (<1600 mg/dl), low steroid exposure (<1225 cDDD) or non-responders less likely to flare
- Early HCQ use and lower initial steroid exposure linked to improved survival
Observational data: associations, not effects.
Further findings
Correlation of drug cDDD with ALT
- AZA cDDD and HCQ cDDD showed a weak positive correlation with ALT
Subtype differences
- Fibrotic subtype associated with older age, renal impairment and lower flare risk; higher HCQ dosage and anaemia increased flare risk
- Proliferative disease showed greater responsiveness to immunosuppressive therapy but shorter median time to first relapse
Original publication
Cumulative defined daily doses enable objective assessment of treatment intensity and outcome prediction in immunoglobulin G4-related disease and its subtypes. Rheumatology (Oxford). Published 25 March 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →