CVID Associated with Systemic Amyloidosis

Area of Science:

• Immunology
• Nephrology
• Internal Medicine

Background:

• Common variable immunodeficiency (CVID) is a frequent primary immune deficiency characterized by recurrent infections, gastrointestinal issues, autoimmunity, and increased malignancy risk.
• Secondary amyloidosis is an infrequent complication of CVID, often associated with chronic inflammation.
• Delayed diagnosis and inadequate treatment of CVID can exacerbate chronic inflammation, potentially leading to severe complications.

Purpose of the Study:

• To report an unusual case of secondary amyloidosis in a patient with Common variable immunodeficiency (CVID).
• To highlight the importance of timely diagnosis and appropriate immunoglobulin replacement therapy in managing CVID.
• To emphasize the need to consider amyloidosis in the differential diagnosis of CVID patients with declining IgG levels and renal complications.

Main Methods:

• Case report of a 27-year-old male diagnosed with CVID presenting with sinopulmonary infections and chronic diarrhea.
• Treatment involved intravenous immunoglobulin (IVIg) and trimethoprim-sulfamethoxazole prophylaxis.
• Monitoring revealed declining IgG levels despite therapy, leading to investigations for proteinuria and kidney biopsy.

Main Results:

• The patient was diagnosed with CVID and treated with IVIg and antibiotics.
• Despite treatment, IgG levels progressively declined, and massive proteinuria was detected.
• Kidney biopsy confirmed the presence of AA type amyloid, indicating secondary amyloidosis.

Conclusions:

• Delayed diagnosis of CVID and suboptimal immunoglobulin replacement therapy contributed to chronic inflammation and the development of secondary amyloidosis.
• Regular monitoring for infection control and adequacy of immunoglobulin therapy is essential for CVID patients.
• Amyloidosis should be considered in the differential diagnosis for CVID patients presenting with renal impairment.