IgG4-related periaortitis presenting as left flank pain

Area of Science:

• Rheumatology and Immunology
• Vascular Inflammation
• Systemic Autoimmune Diseases

Background:

• Periaortitis, an inflammatory condition affecting the aorta, can present with diverse symptoms.
• Immunoglobulin G4-related disease (IgG4-RD) is a rare, fibroinflammatory condition with multisystemic manifestations.
• Accurate diagnosis of IgG4-RD requires a comprehensive approach due to its varied presentation.

Observation:

• A patient presented with left flank pain, initially suggestive of periaortitis.
• The clinical presentation prompted further investigation leading to a diagnosis of IgG4-related disease.
• Serum IgG4 levels were noted to be potentially normal, complicating the diagnostic process.

Findings:

• Diagnosis of IgG4-related disease (IgG4-RD) was established through clinical, serological, radiological, and pathological correlation.
• The case highlights the diagnostic challenges associated with IgG4-RD, emphasizing the need for integrated assessment.
• Normal serum IgG4 levels do not exclude the diagnosis of IgG4-RD.

Implications:

• Early and accurate diagnosis of IgG4-related disease is essential for effective management.
• Immunosuppression is the cornerstone of treatment for IgG4-related disease, requiring long-term management.
• Regular rheumatology input is vital for monitoring and managing patients with chronic IgG4-related disease.