Autoinflammatory & rare systemic diseases

Outcome and risk factors for progression in IgG4-related disease under watchful waiting

Clin Exp RheumatolPublished Retrospective cohort study

Authors' conclusion

IgG4-RD patients with internal organ involvement exhibit a higher rate of disease progression under watchful waiting. Paranasal sinus involvement, high RI, high Eos%, and high IgE levels were identified as potential risk factors for disease progression.

Study context

Population
Immunoglobulin G4-related disease (IgG4-RD) patients managed with the watchful waiting strategy
Participants
40 patients
Exposure
IgG4-RD without vs with internal organ involvement under watchful waiting
  • WOIn = 26reference
  • WIn = 14
Outcome
Disease progression under watchful waiting
Methodological detailslogistic and Cox regression, Kaplan-Meier analysis
  • Logistic and Cox regression
  • Kaplan-Meier analysis

Main finding

As reported in the abstract

Progression

WOI

5

events

WI

7

events

P = 0.048

Progression overall
12/40

Observational data: associations, not effects.

Further findings

Risk factors for progression in WOI

Paranasal sinus involvement, high response index (RI), elevated eosinophil percentage (Eos%) and high IgE levels were significant risk factors for progression

Timing of progression

All progression events occurred within the first year of follow-up; estimated hazard function peaked at 6 to 8 months and declined thereafter

Original publication

Outcome and risk factors for progression in IgG4-related disease under watchful waiting. Clin Exp Rheumatol. Published 23 April 2026. doi:10.55563/clinexprheumatol/iusglt

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