Renal AA amyloidosis in a patient with hereditary complete complement C4 deficiency

• Main Authors: Imed Helal, Rym Goucha, Fethi Ben Hamida, Fethi Elyounsi, Hedi Ben Maiz, Adel Kheder
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2011-01-01
• Series: Saudi Journal of Kidney Diseases and Transplantation
• Online Access: http://www.sjkdt.org/article.asp?issn=1319-2442;year=2011;volume=22;issue=5;spage=1008;epage=1011;aulast=Helal

Hereditary complete C4 deficiency has until now been reported in 30 cases only. A disturbed clearance of immune- complexes probably predisposes these individuals to systemic lupus erythematosus, other immune- complex diseases and recurrent microbial infections. We present here a 20- year- old female with hereditary complete C4 deficiency. Renal biopsy demonstrated renal AA amyloidosis. This unique case further substantiates that deficiency of classical pathway components predisposes to the development of recurrent microbial infections and that the patients may develop AA amyloidosis. Furthermore, in clinical practice, the nephrotic syndrome occurring in a patient with hereditary complete complement C4 deficiency should lead to the suspicion of renal AA amyloidosis.