PDF(Pubmed)
Abstract：
Granulomatous tubulointerstitial nephritis (GTIN) attributed to early onset sarcoidosis is an ultrarare finding in an allograft kidney biopsy. We present the case of a young man with allograft dysfunction who had GTIN upon biopsy. We performed a thorough case review based on recovered records from early childhood and reassessed genetic testing results. We revised his underlying diagnosis from cryopyrin-associated periodic syndrome to early-onset sarcoidosis with wild-type NOD2 and established a rationale to use the interleukin-6 (IL-6) receptor blocker tocilizumab (TCZ). This suppressed his inflammatory disease and stabilised kidney function. We performed a literature review related to the emerging role of IL-6 pathway blockade in kidney transplantation. We identified 18 reports with 417 unique patients treated with TCZ for indications including HLA-desensitisation, transplant immunosuppression induction, treatment of chronic antibody-mediated rejection, and treatment of subclinical rejection. Both TCZ and the direct IL-6 inhibitor clazakizumab are being studied in ongoing randomised control trials.
摘要：
归因于早发性结节病的肉芽肿性肾小管间质性肾炎（GTIN）是同种异体移植肾活检中的超发现。我们介绍了一名患有同种异体移植功能障碍的年轻人，该男子在活检时患有GTIN。我们根据从儿童早期恢复的记录进行了彻底的病例审查，并重新评估了基因检测结果。我们将他的潜在诊断从冷冻比林相关的周期性综合征修改为野生型NOD2的早发性结节病，并建立了使用白介素6（IL-6）受体阻滞剂托珠单抗（TCZ）的基本原理。这抑制了他的炎性疾病并稳定了肾功能。我们进行了有关IL-6通路阻断在肾移植中的新作用的文献综述。我们确定了18例报告，其中417例患者接受TCZ治疗的适应症包括HLA脱敏，移植免疫抑制诱导，治疗慢性抗体介导的排斥反应，和亚临床排斥反应的治疗。TCZ和直接IL-6抑制剂clazakizumab都在正在进行的随机对照试验中进行研究。
