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Chronological change of renal pathological findings in the proliferative glomerulonephritis with monoclonal IgG deposits considered to have recurred early after kidney transplantation

机译:肾移植后早期复发的具有单克隆IgG沉积物的增生性肾小球肾炎的肾脏病理变化按时间顺序变化

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摘要

Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits (PGNMID) is a rare disease that recently became recognized. Its pathological findings are characterized by the deposition of a single heavy chain subclass and a single light chain isotype. PGNMID has been proven to recur in renal allografts. Herein, the authors describe the case of a 46-year-old man who presented with nephrotic syndrome and progressive kidney injury following kidney transplantation. One month after transplantation, his clinical condition stabilized; however, the protocol biopsy showed depositions of IgG and complement on the glomeruli by immunofluorescence staining. Electron microscopy (EM) revealed granular electron-dense deposits (EDD) in the mesangium. Thereafter, renal biopsy was repeated because his proteinuria level increased. Proliferative glomerulonephritis, mainly in the mesangium, with IgG and complement deposits and mesangial and subendothelial EDD were observed; however, the pathological diagnosis was difficult. Renal dysfunction then became apparent, and renal biopsy was performed again 4 years and 10 months after kidney transplantation. Glomerular deposits on a single IgG subclass and a single light chain isotype (IgG3 kappa) with membranoproliferative features were observed. Abundant subendothelial EDD were detected on EM. Finally, the patient was diagnosed with PGNMID. Since it seemed that PGNMID had already developed at 1 month after transplantation, we considered recurrent PGNMID case in the allograft. The treatment for PGNMID has not been established yet, and even in this case, the graft function was eventually lost. For improving renal prognosis, early diagnosis and further investigation on the treatment are necessary.
机译:具有单克隆免疫球蛋白G(IgG)沉积物(PGNMID)的增生性肾小球肾炎是一种罕见的疾病,最近已得到认可。其病理发现的特征在于单个重链亚类和单个轻链同种型的沉积。 PGNMID已被证明可在肾脏同种异体移植中复发。在此,作者描述了一个46岁的男性,该男性在肾脏移植后出现肾病综合征并进行性肾脏损伤的情况。移植后一个月,他的临床情况稳定下来。然而,方案活检显示免疫荧光染色显示肾小球上有IgG和补体沉积。电子显微镜(EM)显示了肾小球系膜中的颗粒状电子致密沉积物(EDD)。此后,由于其蛋白尿水平升高,再次进行了肾脏活检。观察到增生性肾小球肾炎,主要存在于肾小球系膜中,并伴有IgG和补体沉积物以及肾小球膜和内皮下EDD。但是,病理诊断很困难。然后,肾功能不全变得明显,在肾移植后4年和10个月再次进行肾活检。观察到肾小球沉积在单个IgG亚类和具有膜增生性特征的单个轻链同种型(IgG3 kappa)上。在EM上检测到丰富的内皮下EDD。最终,患者被诊断出PGNMID。由于似乎PGNMID在移植后1个月就已经发展,因此我们考虑了同种异体移植中PGNMID复发的情况。 PGNMID的治疗方法尚未建立,即使在这种情况下,移植物功能最终也会丧失。为了改善肾脏的预后,必须及早诊断并进一步研究治疗方法。

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