IgG4-related disease: a complex under-diagnosed clinical entity

Area of Science:

• Rheumatology
• Immunology
• Pathology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a challenging fibro-inflammatory condition.
• Underdiagnosis is common due to its varied clinical presentations and clinician unfamiliarity.
• IgG4-RD often presents as tumor-like masses and tissue-destructive lesions.

Purpose of the Study:

• To increase clinician awareness of IgG4-related disease.
• To review the diagnostic algorithm for IgG4-RD.
• To summarize current understanding of IgG4-RD pathogenesis and treatment.

Main Methods:

• Review of clinical manifestations, histopathology, and serological findings.
• Discussion of the immunological role of IgG4.
• Summary of current treatment modalities, including glucocorticoids and immunomodulators.

Main Results:

• Diagnostic criteria include lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis, and elevated serum IgG4 levels.
• IgG4's role is complex, potentially acting as a pathogen, anti-inflammatory agent, or rheumatoid factor.
• Relapse is common with prolonged glucocorticoid therapy, leading to increased use of immunomodulatory agents.

Conclusions:

• Accurate and timely diagnosis of IgG4-RD is crucial for preventing disease progression.
• Increased awareness and understanding of diagnostic criteria can improve patient outcomes.
• Effective management strategies are evolving, balancing efficacy with long-term safety.