Multimodality Imaging Features of Immunoglobulin G4-related Vessel Involvement

Area of Science:

• Immunology
• Vascular Medicine
• Rheumatology

Background:

• Immunoglobulin 4 (IgG4)-related disease is a chronic fibroinflammatory condition.
• Vascular system involvement, including large and medium vessels, is increasingly recognized in IgG4-related disease.
• Manifestations include aortitis and periaortitis, potentially leading to aneurysm and dissection.

Purpose of the Study:

• To discuss the varied clinical presentations and imaging features of IgG4-related large vessel involvement.
• To highlight the importance of considering IgG4-related large vessel involvement in patients with known or suspected IgG4-related disease.
• To provide an overview of diagnostic considerations and management strategies.

Main Methods:

• Review of clinical, imaging, serum, and pathologic findings associated with IgG4-related large vessel involvement.
• Discussion of typical organ involvement in IgG4-related disease (lacrimal/salivary glands, thyroid, pancreas, etc.).
• Consideration of diagnostic criteria and differential diagnoses.

Main Results:

• IgG4-related large vessel involvement presents with diverse imaging features due to chronic inflammation and fibrosis.
• Aneurysm formation and dissection are potential complications of IgG4-related aortitis and periaortitis.
• Diagnosis requires multidisciplinary correlation of various findings.

Conclusions:

• IgG4-related large vessel involvement is a significant manifestation requiring clinical vigilance.
• Early diagnosis and appropriate management, potentially including corticosteroids or rituximab, are essential.
• Understanding the spectrum of vascular involvement aids in patient care.