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

Abstract

Objectives: IgG4-related disease (IgG4-RD) is a multisystem fibro-inflammatory disorder for which glucocorticoids (GC) represent the initial therapeutic intervention. Second-line treatments are not currently codified. The use of dupilumab, an inhibitor of interleukin (IL)-4 and IL-13 signalling, has recently been described as a potential alternative treatment. The aim of this study was to describe the characteristics of patients IgG4-RD treated with dupilumab and to evaluate its impact on disease control.

Methods: We conducted a French multicentre retrospective study based on standardised questionnaires distributed to physicians via professional networks. The patients included had to meet 2019 ACR/EULAR criteria or the comprehensive diagnostic criteria for IgG4-RD, and to have been treated with dupilumab. Efficacy was assessed using the IgG4-Related Disease Responder Index.

Results: A total of seven patients were included in the study. The rationale for initiating dupilumab was based on the presence of severe asthma, nasosinusal polyps and/or atopic dermatitis. A complete response was observed in three patients, while four patients displayed a partial response. In all cases, GC sparing was achieved. A review of the literature revealed nine out of the ten cases reported where a partial or complete reduction of the disorder was documented, along with GC sparing.

Conclusions: 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.