Tamura Tomomi, Unagami Kohei, Okumi Masayoshi, Kakuta Yoichi, Horita Shigeru, Ishida Hideki, Koike Junki, Honda Kazuho, Tanabe Kazunari, Nitta Kosaku
Department of Nephrology, Tokyo Women's Medical University, Tokyo, Japan.
Department of Urology, Tokyo Women's Medical University, Tokyo, Japan.
Nephrology (Carlton). 2018 Jul;23 Suppl 2:76-80. doi: 10.1111/nep.13280.
Proliferative glomerulonephritis with monoclonal immunoglobulin (Ig)G deposits (PGNMID) is a rare disease with a treatment that is not well established. Several cases of recurrent PGNMID after kidney transplantation have been documented, but almost all cases reported symptoms such as elevated serum creatinine and/or urinary protein levels; subsequently, episode biopsies were performed and a diagnosis was made. This is the case of a 27-year-old man who underwent living-donor kidney transplantation. The aetiology of renal failure was membranoproliferative glomerulonephritis type III, which had been diagnosed at the age of 9 years. Protocol biopsy performed on postoperative day 62 revealed isolated granular C3 deposits in the glomerular capillaries and mesangium. We reviewed the native kidney biopsy and confirmed IgG3 deposition alone, with strong glomerular staining for lambda light chains and negative staining for kappa light chains. Accordingly, we re-diagnosed the aetiology of his renal failure as PGNMID and suspected recurrent PGNMID in the early stage; therefore, we administered plasma exchange therapy. Thereafter, protocol biopsies were performed twice, which revealed persistent isolated C3 deposition; therefore, we made a diagnosis of recurrent PGNMID or C3 glomerulonephritis. Currently, the patient has normal renal function, with negative urine findings for >1 year. Here, we present the histological findings of consecutive allograft biopsies performed in this patient.
伴有单克隆免疫球蛋白(Ig)G沉积的增殖性肾小球肾炎(PGNMID)是一种罕见疾病,其治疗方法尚未完全确立。肾移植后复发性PGNMID的病例已有多例报道,但几乎所有病例均出现血清肌酐升高和/或尿蛋白水平升高等症状;随后进行了发作期活检并作出诊断。本文报道一例27岁接受活体供肾肾移植的男性患者。肾衰竭的病因是III型膜增生性肾小球肾炎,9岁时已确诊。术后第62天进行的方案活检显示肾小球毛细血管和系膜中有孤立的颗粒状C3沉积。我们复查了原肾活检,证实仅有IgG3沉积,λ轻链肾小球染色强,κ轻链染色阴性。因此,我们将其肾衰竭病因重新诊断为PGNMID,并怀疑早期复发PGNMID;因此,我们给予了血浆置换治疗。此后,进行了两次方案活检,均显示持续存在孤立的C3沉积;因此,我们诊断为复发性PGNMID或C3肾小球肾炎。目前,患者肾功能正常,尿检查结果阴性已超过1年。在此,我们展示该患者连续进行的同种异体移植肾活检的组织学结果。