[IgG4-related diseases: A comprehensive review]

Area of Science:

• Immunology
• Pathology
• Rheumatology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a chronic, progressive inflammatory disorder affecting multiple organs.
• Characterized by IgG4-positive plasmacytes and fibrosis, its exact pathogenic mechanisms remain unclear.
• Abnormal regulation of acquired and adaptive immunity is currently implicated in IgG4-RD pathogenesis.

Purpose of the Study:

• To summarize current research progress on IgG4-related disease.
• To review the pathogenesis, clinical features, diagnosis, and treatment of IgG4-RD.

Main Methods:

• Literature review of research on IgG4-related disease.
• Synthesis of findings on pathogenesis, clinical presentation, diagnostic approaches, and therapeutic interventions.

Main Results:

• IgG4-RD presents with diverse clinical manifestations, affecting any tissue or organ.
• Commonly presents as inflammatory pseudotumor or diffuse enlargement, often misdiagnosed.
• Glucocorticoid-based treatments show efficacy, but prognosis can be poor due to variable symptoms.

Conclusions:

• Further research is needed to fully elucidate the pathogenesis of IgG4-RD.
• Accurate diagnosis and management are crucial given the variable presentation and potential for poor prognosis.
• Understanding treatment responses, particularly to glucocorticoids, remains an area of focus.