Exploring the intersection: adult orbital xanthogranulomatous disease and IgG4-related disease – report of 3 cases and literature review

Key Points

• The study aims to clarify the relationship and distinguishing features between AOXD and IgG4-related disease.
• Reported three case studies of adult patients with AOXD and suspected IgG4-related disease.
• Conducted a literature review to assess shared characteristics and diagnostic challenges.
• Recommended comprehensive assessment of serum IgG4 and plasma cell counts.
• Identified overlapping clinical and histopathologic features between AOXD and IgG4-ROD.
• Emphasized the need for careful evaluation to avoid misclassification.
• Noted that corticosteroids are effective, with some cases benefiting from steroid-sparing agents.

Abstract