IgG4-related Disease: a diagnostic challenge

Area of Science:

• Immunology
• Rheumatology
• Pathology

Background:

• Immunoglobulin IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition.
• It can affect virtually any organ, often presenting with tumor-like masses.

Observation:

• A 38-year-old male presented with chronic lumbar pain, edema, and abdominal discomfort.
• Physical examination revealed hepatomegaly and significant leg/scrotal edema.
• Imaging showed a large infrarenal aortic and iliac artery mass compressing the inferior vena cava.

Findings:

• Histopathological examination of a peri-aortic mass biopsy confirmed IgG4-RD.
• Laboratory workup showed anemia but no other significant abnormalities.

Implications:

• This case underscores the diverse clinical presentations of IgG4-RD.
• Early diagnosis and treatment with glucocorticoids can lead to favorable outcomes and mass remission.