Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Clin J Am Soc Nephrol. 2011 Jan;6(1):122-32. doi: 10.2215/CJN.05750710. Epub 2010 Sep 28.
Proliferative GN with monoclonal IgG deposits (PGNMID) is a newly described entity resembling immune complex GN. Its potential to recur in the allograft is undefined.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The first cases of recurrent PGNMID in the allograft are reported.
The cohort includes four Caucasians (3 women, 1 man) with a mean age 58.5 years. No patient had M spike or hematologic malignancy. Recurrence was first documented by biopsy at a mean of 3.8 months posttransplant for indications of renal insufficiency in four patients, proteinuria in three patients, and microhematuria in three patients. Monoclonal IgG deposits (3 IgG3κ and 1 IgG3λ) in the transplants had identical heavy- and light-chain isotypes as in the native kidneys. In two patients, a pattern of endocapillary GN was identified in the native and transplant biopsies, whereas two patients with membranoproliferative GN in the native kidney developed endocapillary or mesangial GN in the transplant. Recurrence was treated with combined high-dose prednisone plus rituximab (n = 3) or plus cyclophosphamide (n = 1). After a mean posttransplant follow-up of 43 months, all four patients achieved reduction in proteinuria and three had reduction in creatinine. Repeat biopsies showed reduced histologic activity after treatment.
PGNMID can recur in the transplant despite the absence of a serum M spike. Recurrence is heralded by proteinuria, hematuria, and allograft dysfunction and manifests diverse histologic patterns. Although the pathogenesis remains unknown, early immunosuppressive therapy appears to stabilize the course.
具有单克隆 IgG 沉积的增生性肾小球肾炎(PGNMID)是一种新描述的类似于免疫复合物性肾小球肾炎的实体。其在移植物中复发的可能性尚未确定。
设计、设置、参与者和测量:报告了首例移植后 PGNMID 复发的病例。
该队列包括 4 名高加索人(3 名女性,1 名男性),平均年龄 58.5 岁。没有患者有 M 峰或血液系统恶性肿瘤。4 名患者因肾功能不全、3 名患者因蛋白尿和 3 名患者因镜下血尿而首先通过活检确诊复发,移植后平均 3.8 个月。移植中的单克隆 IgG 沉积(3 个 IgG3κ 和 1 个 IgG3λ)与原肾中的重链和轻链同种型完全相同。在 2 名患者中,在原肾和移植活检中均发现了毛细血管内肾小球肾炎的模式,而在原肾中患有膜增生性肾小球肾炎的 2 名患者在移植中发展为毛细血管内或系膜肾小球肾炎。复发采用大剂量泼尼松联合利妥昔单抗(n = 3)或联合环磷酰胺(n = 1)治疗。在平均移植后随访 43 个月后,所有 4 名患者的蛋白尿减少,3 名患者的肌酐减少。重复活检显示治疗后组织学活动减少。
尽管血清中没有 M 峰,但 PGNMID 仍可在移植中复发。复发的标志是蛋白尿、血尿和移植物功能障碍,并表现出多种组织学模式。尽管发病机制尚不清楚,但早期免疫抑制治疗似乎可以稳定病情。