[IgG4-related disease - a case report].

Area of Science:

• Immunology
• Rheumatology
• Endocrinology

Background:

• Immunoglobulin G4-related disease (IgG4-RD) is a multi-organ inflammatory condition with non-specific symptoms, often delaying diagnosis.
• Diagnostic criteria include clinical presentation, elevated serum IgG4 levels, and characteristic IgG4-positive plasma cell infiltration on histopathology.
• First-line treatment typically involves oral glucocorticoids.

Observation:

• A 38-year-old woman with a history of Hashimoto disease, chronic sinusitis, and hepatitis presented with chronic eyelid swelling.
• Initial investigations excluded Graves' orbitopathy and systemic vasculitis; however, MRI revealed orbital and lacrimal gland abnormalities.
• Elevated serum IgG4 levels and characteristic IgG4-positive plasma cells in sinonasal tissue confirmed the diagnosis of IgG4-RD.

Findings:

• The patient initially received ineffective oral methylprednisolone.
• Subsequent treatment with methylprednisolone and azathioprine resulted in a significant reduction of symptoms.
• This case highlights the importance of considering IgG4-RD in complex inflammatory presentations.

Implications:

• IgG4-RD requires a high index of suspicion due to its diverse clinical manifestations.
• Combined immunosuppressive therapy may be effective in managing refractory IgG4-RD.
• This case underscores the need for comprehensive diagnostic workups for chronic inflammatory conditions.