Author/Authors :
Helal, Imed Charles Nicolle Hospital - Department of Internal Medicine A, Laboratory of Kidney Pathology 02, Tunisia , Goucha, Rym Charles Nicolle Hospital - Department of Internal Medicine A, Laboratory of Kidney Pathology 02, Tunisia , Ben Hamida, Fethi Charles Nicolle Hospital - Department of Internal Medicine A, Laboratory of Kidney Pathology 02, Tunisia , Elyounsi, Fethi Charles Nicolle Hospital - Department of Internal Medicine A, Laboratory of Kidney Pathology 02, Tunisia , Ben Maiz, Hedi Charles Nicolle Hospital - Department of Internal Medicine A, Laboratory of Kidney Pathology 02, Tunisia , Kheder, Adel Charles Nicolle Hospital - Department of Internal Medicine A, Laboratory of Kidney Pathology 02, Tunisia
Abstract :
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.
