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Management of IgG4-Related Disease
Gabriela Hernández-Molina1, Brian Uriel Anaya-Macías1, Eduardo Martín-Nares2
1Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Belisario Domínguez Sección XVI, Tlalpan, Mexico City, 14080, Mexico.
Current Rheumatology Reports
|May 7, 2026
Summary
IgG4-related disease (IgG4-RD) management is evolving beyond glucocorticoids. B-cell targeted therapies like rituximab and inebilizumab offer effective, steroid-sparing options for this chronic fibroinflammatory condition.
Area of Science:
- Immunology
- Rheumatology
- Pharmacology
Background:
- IgG4-related disease (IgG4-RD) is a chronic, immune-mediated fibroinflammatory condition affecting multiple organs.
- Glucocorticoids are standard for IgG4-RD but have high relapse rates and toxicity.
- Steroid-sparing and targeted therapies are crucial for effective IgG4-RD management.
Purpose of the Study:
- To review current evidence on pharmacological and non-pharmacological management of IgG4-RD.
- To propose a practical, individualized treatment approach for IgG4-RD.
- To highlight emerging therapies for IgG4-RD.
Main Methods:
- Comprehensive literature review of IgG4-RD management strategies.
- Analysis of clinical trial data and expert experience.
- Synthesis of evidence for current and novel therapeutic agents.
Main Results:
- Glucocorticoids induce remission but often lead to relapses.
- Conventional DMARDs are used as steroid-sparing agents with limited comparative data.
- B-cell targeted therapies, particularly rituximab and inebilizumab, show significant efficacy and enable steroid-sparing remission in IgG4-RD.
- Emerging therapies include other biologics and cell-based treatments, requiring further investigation.
Conclusions:
- IgG4-RD treatment requires an individualized approach considering disease severity and patient factors.
- B-cell-directed therapies are pivotal for effective, steroid-sparing IgG4-RD control.
- Novel targeted agents hold promise for improving long-term outcomes in IgG4-RD.
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