RENAL-TRANSPLANTATION IN SECONDARY SYSTEMIC AMYLOIDOSIS

When renal amyloidosis has progressed to end-stage renal failure, most patients are severely affected by systemic amyloidosis and nephropath y. An alternative to chronic dialysis is renal transplantation. We pre sent a patient with amyloid nephropathy who developed recurrent transp lant amyloidosis. Renal transplantation was performed in 1985 with a l iving related donor. In 1990 the patient developed amyloidosis of the graft with membranous nephropathy and tubular acidosis due to hyporeni nemic hypoaldosteronism. Secondary amyloidosis has been reported to in volve glandular organs inducing hypothyroidism, hypocortisolism and hy poaldosteronism. Cyclosporine has been reported to induce hyporeninemi c hypoaldosteronism and tubular acidosis, but not hypocortisolism and hypothyroidism. Progression of the amyloidosis of the graft was confir med by a renal biopsy in 1993. Data published in the literature indica te that the survival rate of amyloidotic graft recipients is worse tha n those of non-amyloidotic graft recipients. This was confirmed in an analysis of the current CTS data which showed an impaired survival rat e at 5-yr of 66 +/- 4% (+/- SE) for patients with amyloidosis (n = 413 ) as compared with 86 +/- 1% (p < 0.0001) for patients with glomerulon ephritis and 84 +/- 1 (p < 0.01) for patients with polycystic kidney d isease. Graft survival after 5 years was 55 +/- 4% in patients with am yloidosis as compared with 63 +/- 1% (p = 0.02) in patients with glome rulonephritis and 68 +/- 1% in patients with polycystic kidney disease . Graft survival was improved in amyloidotic patients treated with cyl osporine as compared with patients on steroids and azathioprine (55 +/ - 4% vs. 38 +/- 8%, p < 0.05). It is concluded that renal transplantat ion is the renal replacement therapy of choice for patients with AA-ty pe amyloidosis although the overall patient survival is impaired in co mparison with patients with other diseases.