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

**Authors:** Andrés González-García, Grisell Starita-Fajardo, David Lucena López, María Pilar Iranzo Alcolea, María López-Paraja, Mercedes Peña-Rodríguez, Francisco Lirola Sánchez, María Sánchez, Adrián Viteri-Noël, Martin Fabregate-Fuente, Mónica López-Rodríguez, José Luis Calleja-López, Luis Manzano Espinosa

PMC · DOI: 10.3390/jcm14196774 · 2025-09-25

## 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.

## Key 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.

## Linked entities

- **Diseases:** IgG4-related disease (MONDO:0017287), IgG4-RD (MONDO:0017287)

## Full-text entities

- **Diseases:** inflammatory (MESH:D007249), IgG4-RD (MESH:D000077733), fibro- (MESH:D009810)
- **Chemicals:** rituximab (MESH:D000069283), steroid (MESH:D013256)

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Source: https://tomesphere.com/paper/PMC12525221