Maria Fareri Children's Hospital, Westchester Medical Center, New York Medical College, Valhalla, NY, USA.
Department of Surgery, Clinical Research Unit, Westchester Medical Center, Valhalla, NY, USA.
Pediatr Nephrol. 2022 Jul;37(7):1667-1673. doi: 10.1007/s00467-021-05341-z. Epub 2021 Nov 18.
Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis is a rare condition in pediatric patients. Little is known about practice patterns and outcomes of pediatric transplant patients. The purpose of our study was to examine differences in patient characteristics, immunosuppression, and long-term graft outcomes between ANCA and non-ANCA vasculitis recipients.
We used the Scientific Registry of Transplant Recipients to evaluate pediatric ANCA vasculitis recipients between the ages of 1 and 22 years old from 1991 to 2017 and compared them to non-ANCA vasculitis patients during the same time cohort in the USA.
A total of 26,431 transplant recipients were identified, of these, 337 with ANCA vasculitis. Mean 1-year eGFR was 62.46 and 64.92 ml/min/1.73 m (p = 0.002), and mean 5-year eGFR was 57.95 and 59.38 ml/min/1.73 m (p = 0.18) between the non-ANCA and ANCA groups, respectively. Five-year graft survival was similar in both groups (non-ANCA 75.5 vs. ANCA 78.6%; p = 0.19). Of those with graft loss within the ANCA group, only 0.6% was secondary to disease recurrence (p = 0.14).
Kidney transplant is a safe treatment modality for children with ANCA-related kidney failure. ANCA patients have comparable graft survival when compared to the general transplant population with a low risk of recurrence. Thymoglobulin was used in a higher proportion within the ANCA group compared to the non-ANCA group. Tacrolimus, mycophenolate mofetil/mycophenolic acid, and steroids were the predominant maintenance immunosuppression used in both groups. A higher resolution version of the Graphical abstract is available as Supplementary information.
抗中性粒细胞胞质抗体（ANCA）血管炎在儿科患者中较为罕见。对于儿科移植患者的实践模式和结局知之甚少。本研究的目的是检查 ANCA 和非 ANCA 血管炎受者在患者特征、免疫抑制和长期移植物结局方面的差异。
我们使用移植受者科学注册处，评估了 1991 年至 2017 年期间年龄在 1 至 22 岁之间的小儿 ANCA 血管炎受者，并将其与同期美国的非 ANCA 血管炎患者进行了比较。
共确定了 26431 例移植受者，其中 337 例为 ANCA 血管炎。非 ANCA 组和 ANCA 组的 1 年平均 eGFR 分别为 62.46 和 64.92ml/min/1.73m（p=0.002），5 年平均 eGFR 分别为 57.95 和 59.38ml/min/1.73m（p=0.18）。两组 5 年移植物存活率相似（非 ANCA 为 75.5%，ANCA 为 78.6%；p=0.19）。在 ANCA 组中，发生移植物丢失的患者中，仅有 0.6%是疾病复发所致（p=0.14）。
对于 ANCA 相关性肾衰竭的儿童，肾移植是一种安全的治疗方式。与一般移植人群相比，ANCA 患者的移植物存活率相当，复发风险较低。与非 ANCA 组相比，ANCA 组中使用了更高比例的胸腺球蛋白。环孢素、霉酚酸酯/吗替麦考酚酯和皮质类固醇是两组中主要的维持性免疫抑制剂。
