Bajcsi Dóra, Constantinou Kypros, Krenács László, Barabás Zsolt, Molnár Szabolcs, Nyiraty Szabolcs, Ábrahám György, Iványi Béla
I. Belgyógyászati Klinika, Nephrologia-Hypertonia Centrum, Szegedi Tudományegyetem, Általános Orvostudományi Kar Szeged, Korányi fasor 8-10., 6720.
Daganatpatológiai és Molekuláris Diagnosztikai Laboratórium, Szegedi Tudományegyetem, Általános Orvostudományi Kar Szeged.
Orv Hetil. 2018 Sep;159(38):1567-1572. doi: 10.1556/650.2018.31072.
Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits is characterized by granular deposits of monoclonal IgG; histologically it has typically a membranoproliferative or endocapillary pattern, and seen electronmicroscopically there are dense deposits without substructure. Here, we present the case of a 62-year-old Caucasian woman who was admitted with rapidly progressive kidney failure. The patient's status, the laboratory and imaging examinations did not support prerenal, postrenal and - among the intrinsic causes - vascular and tubulointerstitial origin. The proteinuria and dysmorphic microhematuria suggested rapidly progressive glomerulonephritis. Tests for anti-neutrophil cytoplasmic antibodies, anti-glomerular basement membrane, antinuclear antibodies and cryoglobulins were negative, the C3 and C4 levels were normal. The biopsy evaluation diagnosed proliferative glomerulonephritis with monoclonal IgG deposits because of mesangial granular deposits of IgG3-kappa, C3, and C1q, and ultrastructurally electron-dense deposits (incidence in our adult native kidney biopsy series: 0.18%). 31 glomeruli were assessed histologically. 29 glomeruli displayed mild mesangial hypercellularity, 2 glomeruli were globally sclerotic. Crescents were not observed. Mild arteriolar hyalinosis, interstitial fibrosis and tubular atrophy accompanied the glomerular alterations. In the postbiopsy evaluation, paraprotein or multiple myeloma was not detected. Despite the mild histological findings, the kidney failure progressed, and hemodialysis had to be started two weeks after the biopsy. Steroids, cyclophosphamide and rituximab did not affect her kidney function, and she remained on hemodialysis during the follow-up of 39 months. This report presents for the first time proliferative glomerulonephritis with monoclonal IgG deposits as the possible cause of rapidly progressive nephritic syndrome in the absence of pronounced glomerular proliferative, sclerotic or tubulointerstitial lesions. Orv Hetil. 2018; 159(38): 1567-1572.
伴有单克隆免疫球蛋白G（IgG）沉积的增殖性肾小球肾炎的特征是单克隆IgG呈颗粒状沉积；组织学上其典型表现为膜增生性或毛细血管内增生性模式，电镜下可见无亚结构的致密沉积物。在此，我们报告一例62岁的白种女性患者，因快速进展性肾衰竭入院。患者的病情、实验室及影像学检查不支持肾前性、肾后性病因，在内在病因中也不支持血管性和肾小管间质性病因。蛋白尿和畸形性镜下血尿提示快速进展性肾小球肾炎。抗中性粒细胞胞浆抗体、抗肾小球基底膜抗体、抗核抗体及冷球蛋白检测均为阴性，C3和C4水平正常。活检评估诊断为伴有单克隆IgG沉积的增殖性肾小球肾炎，原因是IgG3-κ、C3和C1q在系膜区呈颗粒状沉积，超微结构显示为电子致密沉积物（在我们的成人原发性肾活检系列中的发生率为0.18%）。组织学评估了31个肾小球。29个肾小球显示轻度系膜细胞增多，2个肾小球全球硬化。未观察到新月体。轻度小动脉玻璃样变、间质纤维化和肾小管萎缩伴随肾小球改变。活检后评估未检测到副蛋白或多发性骨髓瘤。尽管组织学表现轻微，但肾衰竭仍进展，活检后两周不得不开始血液透析。类固醇、环磷酰胺和利妥昔单抗对其肾功能无影响，在39个月的随访期间她一直接受血液透析。本报告首次提出伴有单克隆IgG沉积的增殖性肾小球肾炎可能是快速进展性肾炎综合征的病因，而无明显的肾小球增殖、硬化或肾小管间质病变。《匈牙利医学周报》。2018年；159（38）：1567 - 1572。
