title: Renal amyloidosis followed more than 5 years: report of 12 cases.
abstract: Renal involvement with amyloidosis is common but causes patient survival to be poor, rarely reaching 5 years. In this study, we retrospectively reviewed clinical and biological characteristics as well as treatments and outcomes of patients with renal amyloidosis followed for more than 5 years. Between 1975 and 2003, 485 patients were diagnosed with renal amyloidosis including only 12 patients who were followed more than 5 years. The six men and six women of mean age 42.4 years (range 18 to 66 years) displayed renal signs of lower limb edema in all cases; hypertension in four cases, proteinuria on urinalysis in all cases with microscopic hematuria in five cases. Biological tests showed nephrotic syndrome in 11 patients, normal renal function in nine patients, and renal failure in three patients whose mean creatinine was 481.6 micromol/L (range 294 to 726). The amyloidosis was AA type in 11 cases and non-AA in one case. An etiologic survey revealed spondylarthropathy in one patient, pulmonary tuberculosis in two patients, chronic bronchitis in three patients, hepatic hydatic cyst in one patient, Mediterranean familial fever in two patients, Crohn's disease in one patient, Hodgkin's lymphoma in one patient, and multiple myeloma in one patient. Specific treatment was initiated with colchicine in seven patients. At a 110-month mean follow-up (range 53 to 153 months), remission of nephrotic syndrome was observed in four cases, progression to chronic renal failure in two patients, and to end-stage renal failure in five cases (range 53 to 196 months), with stabilization of renal function in seven patients. In conclusion, primary amyloid disease should be optimally suppressed in patients with renal involvement. The role of this treatment in remission of renal amyloidosis is not well established. This efficacy of the treatment has been demonstrated in some patients with improved survival.
authors.affiliation: Department of Nephrology and Internal Medicine, Charles Nicolle Hospital, Tunis, Tunisia.
authors.forename: H
authors.initials: H
authors.lastname: Kaaroud
authors.forename: K
authors.initials: K
authors.lastname: Boubaker
authors.forename: S
authors.initials: S
authors.lastname: Béji
authors.forename: E
authors.initials: E
authors.lastname: Abderrahim
authors.forename: F Ben
authors.initials: FB
authors.lastname: Moussa
authors.forename: S
authors.initials: S
authors.lastname: Turki
authors.forename: R
authors.initials: R
authors.lastname: Goucha
authors.forename: H
authors.initials: H
authors.lastname: Hedri
authors.forename: F
authors.initials: F
authors.lastname: El Younsi
authors.forename: A
authors.initials: A
authors.lastname: Kheder
authors.forename: H Ben
authors.initials: HB
authors.lastname: Maiz
mesh_terms: D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000401:mortality / Q000628:therapy*; D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis; D012189:Retrospective Studies; D016019:Survival Analysis; D013997:Time Factors