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

Lancet RheumatolPublished Retrospective cohort study

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

OutcomeMaleFemaleP value
ACR-EULAR classification criteria score at baseline35.0 (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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