Systemic reactive (AA) amyloidosis in the course of common variable immunodeficiency

Patients with primary immunodeficiencies (PID) are currently at low risk for the development of systemic reactive AA amyloidosis. A patient with common variable immunodeficiency and nephrotic syndrome was referred to our Clinic from a Nephrology unit. She had a history of recurrent infections. Monthly intramuscular immunoglobulin substitution treatment was started in 1979, subsequently switched to intravenous route. Between 1984 and 2007, she continued to experience infections and did not have follow-up visits in an Immunology Centre. Replacement therapy was discontinued in 2008 for adverse events related to IVIG infusion; since then she was admitted for recurrent cellulitis, sepsis, and pneumonia. In 2009, the patient developed massive proteinuria and renal failure due to clinically overt reactive systemic AA amyloidosis. Despite the prompt resumption of antibody replacement, adequate IgG levels were not achieved and SAA concentration remained elevated. Delay in diagnosis and inadequate treatment of PIDs results in increased irreversible complications, including AA amyloidosis.