Department of Internal Medicine, Division of Rheumatology, Baskent University Faculty of Medicine, Ankara, Turkey.
Department of Internal Medicine, Baskent University Faculty of Medicine, Ankara, Turkey.
Clin Transplant. 2023 Feb;37(2):e14888. doi: 10.1111/ctr.14888. Epub 2023 Jan 3.
Long-term kidney transplantation (KT) results in patients with familial Mediterranean fever (FMF)-related amyloidosis are not well studied. This study reviewed the long-term survival outcomes of FMF patients who underwent KT.
We compared the outcomes of 31 patients who underwent (KT) for biopsy-proven amyloidosis secondary to FMF with 31 control patients (five with diabetes mellitus and 26 with nondiabetic kidney disease) undergoing KT between 1994 and 2021 at Başkent University Hospital. All data were recorded retrospectively from patients' files.
THE MEDIAN AGE (QUARTILE DEVIATION: QD) at the time of KT in the FMF and control group were 31 (6.7) and 33 (11), respectively. The median follow-up period (QD) after KT was 108 (57) months in the FMF and 132 (72) months in the control group. In the FMF group, graft and patient survivals were 71% and 84% at 5 years and 45% and 48% at 10 years, respectively. In the control group, graft and patient survivals were 79% and 100% at 5 years and 63% and 71% at 10 years, respectively. Patient survival in the FMF group at 5 years was significantly lower than in the control group (p = .045). There was no statistically significant difference between the FMF and control groups in terms of graft and patient survival, and serum creatinine levels at 10 years. All patients were given triple immunosuppressive treatment with cyclosporine, mycophenolate mofetil, and prednisolone. Three patients received anakinra and one received canakinumab in addition to colchicine treatment. One FMF patient also underwent heart transplantation due to AA amyloidosis. Of the FMF patients, 11 died during follow-up.
We have found that the long-term outcome of KT in patients with FMF amyloidosis is numerically worse but not statistically different from the control group. However, short- and long-term complications still need to be resolved.
长期接受肾移植（KT）的家族性地中海热（FMF）相关淀粉样变性患者的结果尚未得到充分研究。本研究回顾了接受 KT 的 FMF 患者的长期生存结果。
我们比较了 31 名接受 KT 的患者（活检证实为 FMF 引起的淀粉样变性）与 31 名对照组患者（5 例糖尿病和 26 例非糖尿病肾病）的结局，这些患者于 1994 年至 2021 年在 Başkent 大学医院接受 KT。所有数据均从患者的档案中回顾性记录。
FMF 组和对照组患者 KT 时的中位年龄（四分位距[QD]）分别为 31（6.7）和 33（11）。FMF 组 KT 后中位随访时间（QD）为 108（57）个月，对照组为 132（72）个月。在 FMF 组，5 年时移植物和患者存活率分别为 71%和 84%，10 年时分别为 45%和 48%。在对照组中，5 年时移植物和患者存活率分别为 79%和 100%，10 年时分别为 63%和 71%。FMF 组患者 5 年时的存活率明显低于对照组（p = 0.045）。FMF 组和对照组在 10 年时移植物和患者存活率以及血清肌酐水平方面无统计学差异。所有患者均接受环孢素、霉酚酸酯和泼尼松龙三联免疫抑制治疗。3 例患者接受阿那白滞素治疗，1 例患者接受卡那单抗治疗，同时接受秋水仙碱治疗。1 例 FMF 患者还因 AA 淀粉样变性接受了心脏移植。在 FMF 患者中，11 例患者在随访期间死亡。
我们发现，FMF 淀粉样变性患者 KT 的长期结果在数值上较差，但与对照组无统计学差异。然而，仍需要解决短期和长期并发症的问题。
