New Developments in the Treatment of IgG4-Related Disease: A Comprehensive Clinical Approach

TL;DR

This paper reviews new treatment options for IgG4-related disease, focusing on steroid alternatives and personalized approaches.

Contribution

The paper provides an updated overview of emerging treatment strategies for IgG4-RD, emphasizing personalized management.

Findings

Rituximab shows efficacy in treating IgG4-RD but lacks consensus on optimal maintenance regimens.

New B-cell–targeted therapies and immunomodulators offer promising personalized treatment options.

Prolonged glucocorticoid use remains a challenge, prompting the need for steroid-sparing alternatives.

Abstract

Immunoglobulin G4–related disease (IgG4-RD) is an uncommon fibro-inflammatory process characterized by the infiltration of tissues and organs and a typically dramatic response to glucocorticoids. Its relapsing–remitting course, multisystemic involvement, and variability in epidemiological and prognostic features pose a significant diagnostic challenge for clinicians. Despite their effectiveness in symptom relief, prolonged glucocorticoid use remains a challenge in IgG4-RD management, prompting the search for steroid-sparing alternatives. Although rituximab has recently demonstrated efficacy in the treatment of IgG4-RD, no consensus exists regarding the optimal maintenance regimen. The emergence of new B-cell–targeted therapies and other immunomodulators represents a promising step toward more personalized treatment approaches. In this review, we provide an updated and integrative overview of the emerging treatment strategies for IgG4-RD, highlighting future directions towards individualized management.