Department of Nephrology and Transplantation Center, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan.
Nephrology (Carlton). 2014 Jun;19 Suppl 3:45-8. doi: 10.1111/nep.12252.
IgA nephropathy (IgAN) is recurrent after transplantation; however, its time of recurrence is unpredictable. To date, factors influencing IgAN recurrence have not been elucidated. We present a case of a 23-year-old man with end-stage renal disease (ESRD) who underwent living-related ABO-identical pre-emptive kidney transplantation (PEKT) using his 57-year-old mother as a donor. IgAN started when the patient was 19 years old, and renal biopsy revealed the usual pathological findings of IgAN. In spite of steroid therapy including steroid pulse and tonsillectomy, the patient developed nephrotic syndrome and progressed to ESRD in 4 years. Protocol biopsy on day 19 following PEKT revealed active recurrent IgAN. Nephrotic-range proteinuria and mild deterioration of kidney function developed regardless of strong immunosuppressive therapy such as steroid pulse, double filtration plasmapheresis and rituximab. We report a case of refractory IgAN that recurred 19 days after transplantation. This case is considered of value to elucidate factors leading to active IgAN recurrence.
IgA 肾病(IgAN)在移植后会复发;然而,其复发时间是不可预测的。迄今为止,影响 IgAN 复发的因素尚未阐明。我们报告了一例 23 岁的终末期肾病(ESRD)患者,他使用 57 岁的母亲作为供体进行了活体相关 ABO 同型预先肾移植(PEKT)。该患者 19 岁时出现 IgAN,肾活检显示 IgAN 的常见病理表现。尽管包括类固醇脉冲和扁桃体切除术在内的类固醇治疗,但该患者在 4 年内发展为肾病综合征并进展为 ESRD。PEKT 后第 19 天的方案活检显示活跃的复发性 IgAN。尽管采用了强烈的免疫抑制治疗,如类固醇脉冲、双重滤过血浆置换和利妥昔单抗,仍出现大量蛋白尿和肾功能轻度恶化。我们报告了一例难治性 IgAN 的病例,该病例在移植后 19 天复发。该病例有助于阐明导致 IgAN 活跃复发的因素。