IgG4-Related disease: From diagnosis to remission

Area of Science:

• Immunology
• Rheumatology
• Pathology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition impacting multiple organs.
• Understanding IgG4-RD requires recognizing diverse clinical presentations, from glandular issues to vascular complications, and both inflammatory and fibrotic aspects.

Purpose of the Study:

• To provide a comprehensive overview of current knowledge on IgG4-RD.
• To address disease mechanisms, diagnostic methods, and therapeutic strategies.
• To highlight existing knowledge gaps and future research directions.

Main Methods:

• Review of current literature on IgG4-RD.
• Analysis of diagnostic criteria integrating clinical, imaging, histopathological, and serological data.
• Evaluation of evolving treatment strategies, including B cell depletion therapy.

Main Results:

• IgG4-RD presents with varied manifestations, necessitating integrated diagnostic approaches.
• B cell depletion therapy (e.g., inebilizumab) has improved treatment options.
• Glucocorticoid-sparing strategies are preferred, but long-term management remains challenging.

Conclusions:

• Effective management of IgG4-RD requires balancing disease control with minimizing immunosuppression risks.
• Further research is crucial for identifying biomarkers, establishing remission criteria, and elucidating etiopathogenesis.
• Targeted therapies are needed to address persistent challenges in IgG4-RD treatment.