National Amyloidosis Centre, University College London, London, UK.
Department of Renal Medicine, Centre for Transplantation, University College London, London, UK.
Nephrol Dial Transplant. 2021 Jan 25;36(2):355-365. doi: 10.1093/ndt/gfaa293.
Outcomes after renal transplantation have traditionally been poor in systemic amyloid A (AA) amyloidosis and systemic light chain (AL) amyloidosis, with high mortality and frequent recurrent disease. We sought to compare outcomes with matched transplant recipients with autosomal dominant polycystic kidney disease (ADPKD) and diabetic nephropathy (DN), and identify factors predictive of outcomes.
We performed a retrospective cohort study of 51 systemic AL and 48 systemic AA amyloidosis patients undergoing renal transplantation. Matched groups were generated by propensity score matching. Patient and death-censored allograft survival were compared via Kaplan-Meier survival analyses, and assessment of clinicopathological features predicting outcomes via Cox proportional hazard analyses.
One-, 5- and 10-year death-censored unadjusted graft survival was, respectively, 94, 91 and 78% for AA amyloidosis, and 98, 93 and 93% for AL amyloidosis; median patient survival was 13.1 and 7.9 years, respectively. Patient survival in AL and AA amyloidosis was comparable to DN, but poorer than ADPKD [hazard ratio (HR) = 3.12 and 3.09, respectively; P < 0.001]. Death-censored allograft survival was comparable between all groups. In AL amyloidosis, mortality was predicted by interventricular septum at end diastole (IVSd) thickness >12 mm (HR = 26.58; P = 0.03), while survival was predicted by haematologic response (very good partial or complete response; HR = 0.07; P = 0.018). In AA amyloidosis, recurrent amyloid was associated with elevated serum amyloid A concentration but not with outcomes.
Renal transplantation outcomes for selected patients with AA and AL amyloidosis are comparable to those with DN. In AL amyloidosis, IVSd thickness and achievement of deep haematologic response pre-transplant profoundly impact patient survival.
在淀粉样变性 A（AA）淀粉样变性和系统性轻链（AL）淀粉样变性中，肾移植后的结局传统上较差，死亡率高且疾病频繁复发。我们旨在比较与常染色体显性多囊肾病（ADPKD）和糖尿病肾病（DN）相匹配的移植受者的结局，并确定预测结局的因素。
我们对 51 例系统性 AL 和 48 例系统性 AA 淀粉样变性患者进行了回顾性队列研究。通过倾向评分匹配生成匹配组。通过 Kaplan-Meier 生存分析比较患者和死亡相关移植物存活率，并通过 Cox 比例风险分析评估预测结局的临床病理特征。
AA 淀粉样变性的未调整死亡相关移植物 1 年、5 年和 10 年存活率分别为 94%、91%和 78%，AL 淀粉样变性分别为 98%、93%和 93%；中位患者存活率分别为 13.1 年和 7.9 年。AL 和 AA 淀粉样变性患者的生存率与 DN 相当，但比 ADPKD 差[风险比（HR）分别为 3.12 和 3.09；P<0.001]。所有组之间的死亡相关移植物存活率相当。在 AL 淀粉样变性中，室间隔末期厚度（IVSd）>12mm（HR=26.58；P=0.03）预测死亡率，而血液学反应（非常好的部分或完全反应；HR=0.07；P=0.018）预测存活率。在 AA 淀粉样变性中，复发性淀粉样蛋白与血清淀粉样 A 浓度升高相关，但与结局无关。
对于 AA 和 AL 淀粉样变性的某些患者，肾移植的结局与 DN 相似。在 AL 淀粉样变性中，IVSd 厚度和移植前达到深度血液学反应显著影响患者的生存。
