IgG4-related disease - focus on digestive system involvement

Area of Science:

• Immunology
• Rheumatology
• Gastroenterology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition.
• Characterized by IgG4-positive plasma cell infiltration, leading to fibrosis and organ dysfunction.
• Key manifestations include autoimmune pancreatitis and sclerosing cholangitis, posing diagnostic challenges.

Purpose of the Study:

• To review the epidemiology, pathogenesis, and organ manifestations of IgG4-related disease.
• To highlight diagnostic challenges and advances in detection.
• To discuss current and emerging treatment strategies.

Main Methods:

• Literature review focusing on IgG4-related disease.
• Analysis of epidemiological data, pathogenesis, clinical presentations, diagnostic criteria, and treatment outcomes.
• Synthesis of information on diagnostic markers, imaging, and therapeutic agents.

Main Results:

• IgG4-RD presents with diverse organ involvement, notably autoimmune pancreatitis and sclerosing cholangitis.
• Diagnosis relies on clinical, imaging, serological (elevated serum IgG4), and histopathological findings.
• Steroids are primary for remission, with DMARDs and rituximab crucial for relapses or resistant cases.

Conclusions:

• Advances in understanding, diagnosis, and treatment have improved IgG4-RD management.
• A multidisciplinary approach is essential for optimizing patient outcomes.
• Biologic therapies offer promising future treatment avenues for IgG4-RD.