Secondary Amyloidosis and Common Variable Immunodeficiency: A Rare Association

Area of Science:

• Nephrology
• Immunology
• Internal Medicine

Background:

• Common variable immunodeficiency (CVID) is a primary immunodeficiency marked by severe antibody deficiency and impaired B cell differentiation.
• Clinical manifestations include recurrent infections, chronic lung disease, gastrointestinal issues, and autoimmunity.
• Long-term intravenous immunoglobulin (IVIG) therapy is standard for CVID management.

Observation:

• A 47-year-old female with CVID and recurrent Campylobacter jejuni bacteremia presented with acute renal failure.
• The patient exhibited anasarca, nephrotic syndrome, and bilateral nephromegaly on CT scan.
• Renal biopsy confirmed amyloidosis with elevated serum amyloid A protein.

Findings:

• The patient was diagnosed with AA amyloidosis secondary to CVID.
• This represents a rare but serious complication of CVID.
• Prompt diagnosis and management are crucial for patient outcomes.

Implications:

• This case underscores the need for vigilance regarding renal complications in CVID patients, irrespective of long-term IVIG treatment.
• Early detection and intervention, including dialysis and adjusted IVIG, can lead to favorable clinical outcomes.
• Further research into the pathogenesis of CVID-associated amyloidosis is warranted.