IgG4-related disease: a clinical perspective

Area of Science:

• Rheumatology
• Immunology
• Pathology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a recently identified fibro-inflammatory disorder with diverse organ involvement.
• Key histopathological findings include IgG4+ plasma cell infiltration, storiform fibrosis, and obliterative phlebitis.
• Pathogenic mechanisms involving CD4+ T and B cells are implicated in organ damage and fibrosis.

Purpose of the Study:

• To provide an updated review of the clinical and therapeutic aspects of IgG4-related disease.
• To highlight diagnostic challenges and approaches for IgG4-related disease.
• To discuss current and emerging treatment strategies for IgG4-RD.

Main Methods:

• Literature review of clinical and therapeutic aspects of IgG4-related disease.
• Analysis of histopathological, laboratory, and radiological diagnostic criteria.
• Evaluation of treatment outcomes with glucocorticoids and rituximab.

Main Results:

• IgG4-RD presents with various organ involvements, including salivary glands, orbits, pancreas, and kidneys.
• Diagnosis requires a combination of histopathology, clinical context, and imaging.
• Glucocorticoids are first-line therapy, while rituximab shows efficacy in refractory or relapsing cases.

Conclusions:

• IgG4-RD is a multi-organ disorder requiring a comprehensive diagnostic approach.
• Effective management strategies include glucocorticoids and B-cell depletion therapy with rituximab.
• Further research into pathogenic mechanisms may lead to novel therapeutic targets.