Plonsky Toder Moran, Tibi Rami, Steinberg Ran, Karram Tony, Hoffman Aharon, Coleman Dawn, Libinson-Zebegret Irina, Yakubov Renata, Eisenstein Israel, Magen Daniella, Pollack Shirley
Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel.
Ruth Rappaport Children's Hospital, Rambam Health Care Campus, Pediatric Nephrology Institute, Haifa, Israel.
Pediatr Nephrol. 2025 Sep 11. doi: 10.1007/s00467-025-06932-w.
Takayasu arteritis (TAK) is a granulomatous large-vessel vasculitis typically affecting young adult females. Pediatric cases are rare, and infantile onset is exceptional. Management relies on immunosuppression, with surgery reserved for severe complications.
We describe a now 5.5-year-old boy diagnosed with TAK at six months of age, presenting with hypertensive encephalopathy and kidney dysfunction. Despite treatment with corticosteroids and anti-TNFα, his kidney function deteriorated, leading to kidney failure and dialysis. At nearly three years of age, he underwent abdominal aorto-aortic bypass and bilateral nephrectomy due to progressive vascular narrowing and refractory hypertension. At age four, he successfully received a deceased-donor kidney transplant. Eighteen months post-transplant, he maintains excellent graft function and shows no signs of TAK recurrence.
This case underscores the complexity of diagnosing and managing infantile TAK with multiorgan involvement. To our knowledge, he is among the youngest reported TAK patients to undergo successful kidney transplantation following major vascular surgery. His course demonstrates the potential for long-term remission and safe transplantation under standard immunosuppression, without continued anti-TNFα therapy. The literature is sparse regarding kidney failure and transplantation in TAK, particularly in infants.
This case highlights key management dilemmas in infantile TAK, including clinical diagnosis, timing of surgery and transplantation, choice of immunosuppression, and long-term monitoring. It emphasizes the importance of a multidisciplinary approach and the need for collaborative research to address knowledge gaps in this rare but complex condition.
大动脉炎（TAK）是一种肉芽肿性大血管血管炎，通常影响年轻成年女性。儿科病例罕见，婴儿期发病更是罕见。治疗依赖免疫抑制，手术仅用于严重并发症。
我们描述了一名现5.5岁的男孩，他在6个月大时被诊断为TAK，表现为高血压脑病和肾功能不全。尽管接受了皮质类固醇和抗TNFα治疗，但其肾功能仍恶化，导致肾衰竭并需要透析。近3岁时，由于进行性血管狭窄和顽固性高血压，他接受了腹主动脉-主动脉旁路手术和双侧肾切除术。4岁时，他成功接受了 deceased-donor 肾移植。移植后18个月，他的移植肾功能良好，无TAK复发迹象。
该病例强调了诊断和管理累及多器官的婴儿期TAK的复杂性。据我们所知，他是报道中接受重大血管手术后成功进行肾移植的最年轻TAK患者之一。他的病程表明在标准免疫抑制下有长期缓解和安全移植的可能性，无需持续抗TNFα治疗。关于TAK患者肾衰竭和移植的文献，尤其是婴儿患者，较为稀少。
该病例突出了婴儿期TAK的关键管理难题，包括临床诊断、手术和移植时机、免疫抑制选择以及长期监测。它强调了多学科方法的重要性以及开展合作研究以填补这一罕见但复杂疾病知识空白的必要性。
