End-stage renal failure due to amyloidosis: outcomes in 490 ANZDATA registry cases

Abstract

Background: There are few reports regarding the long-term renal replacement therapy (RRT) outcomes of amyloidosis. Methods: In this retrospective, multi-centre, multi-country registry analysis, all patients with and without amyloidosis who commenced RRT for end-stage renal failure (ESRF) in Australia and New Zealand between 1963 and 2010 were included. Results: Of 58 422 patients who underwent RRT during the study period, 490 (0.8%) had ESRF secondary to amyloidosis. The median survival of amyloidosis patients on dialysis (2.09 years, 95% CI 1.85–2.32 years) was significantly inferior to that of patients with other causes of ESRF (4.45 years, 95% CI 4.39–4.51 years) (log-rank score 242, P < 0.001). The survival of amyloidosis patients receiving peritoneal dialysis (1.9 years, 95% CI 1.58–2.22) was comparable with those receiving haemodialysis (2.17 years, 95% CI 1.89–2.45) (P = 0.18). Fifty-three (13.8%) amyloidosis patients died of amyloidosis complications. Forty-six patients underwent renal transplantation with first graft survival rates of 45% at 5 years and 26% at 10 years. Nine (16.4%) patients experienced amyloidosis recurrence in their allografts, which led to graft failure in six patients. ESRF patients with amyloidosis experienced inferior median first renal allograft survival (4.55 years, 95% CI 1.96–7.15 versus 10.7 years, 95% CI 10.5–11.0, P = 0.001) and transplant patient survival (6.03 years, 95% CI 2.71–9.36 versus 16.8 years, 95% CI 16.4–17.1, P < 0.001) compared with patients with other causes of ESRF. Respective 10-year patient survival rates were 37 and 69%. Conclusions: Amyloidosis was associated with poor patient survival following dialysis and/or renal transplantation, poor renal allograft survival and a significant incidence of disease recurrence in the allograft. An appreciable proportion of amyloid ESRF patients died of amyloidosis-related complications.