IgG4-related disease: lessons from the first 20 years

Area of Science:

• Rheumatology
• Immunology
• Pathology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition with suspected autoimmune origins.
• Understanding of IgG4-RD pathophysiology and clinical features has advanced significantly since its identification.
• Early intervention is vital to prevent irreversible organ damage from fibrosis.

Purpose of the Study:

• To outline an approach for diagnosing IgG4-RD.
• To highlight the importance of recognizing clinical manifestations and integrating diagnostic data.
• To emphasize the need to differentiate IgG4-RD from other conditions like malignancy.

Main Methods:

• Utilizing the American College of Rheumatology/European League Against Rheumatism Classification Criteria as a diagnostic framework.
• Incorporating clinical, radiological, serological, and histopathological findings.
• Systematically excluding other potential diseases, especially malignancies.

Main Results:

• Glucocorticoids and B cell depletion therapies demonstrate efficacy in inducing remission for most IgG4-RD patients.
• Ongoing clinical trials are defining the optimal therapeutic strategies.

Conclusions:

• Accurate diagnosis of IgG4-RD requires a comprehensive approach integrating diverse data.
• Effective management strategies, including B cell depletion and glucocorticoids, are available.
• Further research is needed to refine treatment protocols and understand the disease's autoimmune basis.