Autoinflammatory & rare systemic diseases
Sex as a predictor of clinical phenotype and determinant of immune response in IgG4-related disease: a retrospective study of patients fulfilling the American College of Rheumatology–European League Against Rheumatism classification criteria
Authors' conclusion
IgG4-related disease is unusual among autoimmune diseases in that it is more likely to affect males than females and to present with a striking sex-dependent organ distribution and degree of B-cell response.
Study context
- Population
- Patients with IgG4-RD fulfilling ACR-EULAR classification criteria, Massachusetts General Hospital Rheumatology Clinic (Boston, MA, USA), January 2008–May 2023of 564 registry participants
- Participants
- 328 patients
- Exposure
- Male vs female patients with IgG4-RD
- Malesn = 226
- Femalesn = 102reference
- Outcome
- The main outcomes were assessed in all participants with available data: differences in IgG4-RD manifestations between male and female patients
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
Sex distribution
- Male:female ratio
- 2.2:1
- General rheumatology clinic population male:female ratio (p<0.001)
- 0.4:1
Observational data: associations, not effects.
Further findings
Age at diagnosis · on average, male patients older at diagnosis
difference 5.5 · P = 0.0031
Median
| Outcome | Male | Female | P value |
|---|---|---|---|
| ACR-EULAR classification criteria score at baseline | 35.0 (IQR 28.0–46.0) | 29.5 (IQR 25.0–39.0) | P = 0.0010 |
Original publication
Sex as a predictor of clinical phenotype and determinant of immune response in IgG4-related disease: a retrospective study of patients fulfilling the American College of Rheumatology–European League Against Rheumatism classification criteria. Lancet Rheumatol. Published 30 May 2024. doi:10. 1016/ s2665-9913(24)00089-4
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