title: Renal AA amyloidosis in a patient with hereditary complete complement C4 deficiency.
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.
authors.affiliation: Department of Internal Medicine A and Laboratory of Kidney Pathology 02, Charles Nicolle Hospital, Tunis, Tunisia. imedhelal@viola.fr
authors.forename: Imed
authors.initials: I
authors.lastname: Helal
authors.forename: Rym
authors.initials: R
authors.lastname: Goucha
authors.forename: Fethi Ben
authors.initials: FB
authors.lastname: Hamida
authors.forename: Fethi
authors.initials: F
authors.lastname: Elyounsi
authors.forename: Hedi Ben
authors.initials: HB
authors.lastname: Maiz
authors.forename: Adel
authors.initials: A
authors.lastname: Kheder
mesh_terms: D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000150:complications*; D001987:Bronchiectasis / Q000150:complications; D003181:Complement C4 / Q000172:deficiency*; D005260:Female; D006801:Humans; D011008:Pneumococcal Infections / Q000150:complications; D018410:Pneumonia, Bacterial / Q000150:complications; D012008:Recurrence; D000685:Serum Amyloid A Protein; D055815:Young Adult
chemical_list: D000682:Amyloid; D003181:Complement C4; D000685:Serum Amyloid A Protein