title: Renal transplantation in secondary systemic amyloidosis.
abstract: When renal amyloidosis has progressed to end-stage renal failure, most patients are severely affected by systemic amyloidosis and nephropathy. An alternative to chronic dialysis is renal transplantation. We present a patient with amyloid nephropathy who developed recurrent transplant amyloidosis. Renal transplantation was performed in 1985 with a living related donor. In 1990 the patient developed amyloidosis of the graft with membranous nephropathy and tubular acidosis due to hyporeninemic hypoaldosteronism. Secondary amyloidosis has been reported to involve glandular organs inducing hypothyroidism, hypocortisolism and hypoaldosteronism. Cyclosporine has been reported to induce hyporeninemic hypoaldosteronism and tubular acidosis, but not hypocortisolism and hypothyroidism. Progression of the amyloidosis of the graft was confirmed by a renal biopsy in 1993. Data published in the literature indicate that the survival rate of amyloidotic graft recipients is worse than those of non-amyloidotic graft recipients. This was confirmed in an analysis of the current CTS data which showed an impaired survival rate at 5-yr of 66 +/- 4% (+/- SE) for patients with amyloidosis (n = 413) as compared with 86 +/- 1% (p < 0.0001) for patients with glomerulonephritis and 84 +/- 1 (p < 0.01) for patients with polycystic kidney disease. Graft survival after 5 years was 55 +/- 4% in patients with amyloidosis as compared with 63 +/- 1% (p = 0.02) in patients with glomerulonephritis and 68 +/- 1% in patients with polycystic kidney disease. Graft survival was improved in amyloidotic patients treated with cylosporine as compared with patients on steroids and azathioprine (55 +/- 4% vs. 38 +/- 8%, p < 0.05). It is concluded that renal transplantation is the renal replacement therapy of choice for patients with AA-type amyloidosis although the overall patient survival is impaired in comparison with patients with other diseases.
authors.affiliation: Department of Nephrology and Rheumatology, Heinrich Heine University, Düsseldorf, Germany.
authors.forename: P
authors.initials: P
authors.lastname: Heering
authors.forename: R
authors.initials: R
authors.lastname: Hetzel
authors.forename: B
authors.initials: B
authors.lastname: Grabensee
authors.forename: G
authors.initials: G
authors.lastname: Opelz
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D018450:Disease Progression; D010505:Familial Mediterranean Fever / Q000150:complications; D006085:Graft Survival; D006801:Humans; D007150:Immunohistochemistry; D007676:Kidney Failure, Chronic / Q000150:complications / Q000473:pathology / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male