Autoinflammatory & rare systemic diseases

Efficacy and glucocorticoid sparing of dupilumab in IgG4-related disease: a case series and literature review

Clin Exp RheumatolPublished Retrospective case series and literature review

Authors' conclusion

These preliminary findings need to be confirmed, but may offer arguments in favour of a possible efficacy of dupilumab in the treatment of IgG4-RD.

Study context

Population
French patients meeting 2019 ACR/EULAR criteria or comprehensive diagnostic criteria for igG4-related disease (IgG4-RD), treated with dupilumab
Participants
7 patients
Outcome
Disease control assessed by the IgG4-Related Disease Responder Index
Methodological detailsmulticentre, standardised questionnaires, Indication for dupilumab: severe asthma, nasosinusal polyps and/or atopic dermatitis
  • Multicentre
  • Standardised questionnaires
  • Indication for dupilumab: severe asthma, nasosinusal polyps and/or atopic dermatitis

Main finding

As reported in the abstract

Response (IgG4-RD Responder Index)
Complete response
3 patients
Partial response
4 patients
Glucocorticoids (GC) sparing achieved
all cases

Observational data: associations, not effects.

Further findings

Literature review: partial or complete reduction of the disorder with GC sparing

Cases with partial or complete reduction and GC sparing
9 of 10 reported cases

Original publication

Efficacy and glucocorticoid sparing of dupilumab in IgG4-related disease: a case series and literature review. Clin Exp Rheumatol. Published 29 November 2024. doi:10.55563/clinexprheumatol/nui493

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