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A case presenting with the possible relationship between myeloperoxidase–antineutrophil cytoplasmic antibody-associated glomerulonephritis and membranous changes of the glomerular basement membrane

机译:髓过氧化物酶-抗中性粒细胞胞浆抗体相关性肾小球肾炎与肾小球基底膜膜性改变之间可能存在关系的病例

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A 72-year-old woman exhibited elevated serum myeloperoxidase–antineutrophil cytoplasmic antibody (MPO-ANCA) levels since 2006. Her serum creatinine (sCr) levels increased from 0.5 to 1.62 mg/dl in a stepwise pattern with proteinuria and hematuria up to January 2011. Renal biopsy indicated global sclerosis (14 %), fibrocellular crescents (28 %), and Swiss cheese-like appearance of the glomerular basement membrane (GBM) on light microscopy. IgG4 staining was negative. Immunofluorescent examination indicated granular staining with IgG and C3 along the GBM. MPO-ANCA-associated glomerulonephritis with membranous nephropathy (MN) was diagnosed. As chronic changes were relatively evident in the renal biopsy specimen without acute augmentation of renal function, immunosuppressive therapy was not administered. Thereafter, rapidly progressive renal dysfunction occurred (sCr, 3.67 mg/dl in May 2011) with proteinuria (~2 g/day), hematuria, and elevated serum MPO-ANCA levels. Therefore, a second renal biopsy was performed in May 2011, indicating global sclerosis (42 %) and cellular crescents (35 %) on light microscopy. Electron microscopy indicated electron-dense deposits in the GBM and mesangial lesions. Steroid therapy was subsequently initiated, and the patient’s renal function partially improved. MPO-ANCA levels decreased to within normal limits and hematuria disappeared. MPO-ANCA-associated glomerulonephritis with MN is a rare dual glomerulopathy. However, complication should be considered when urinary protein appears in large amounts. Secondary MN was suspected due to the lack of IgG4 staining and distribution of electron-dense deposits to the mesangial lesion. Renal dysfunction occurring in a stepwise pattern may be attributed to intermittent augmentation in MPO-ANCA-associated glomerulonephritis.
机译:自2006年以来,一名72岁妇女的血清髓过氧化物酶-抗中性粒细胞胞浆抗体(MPO-ANCA)水平升高。她的血清肌酐(sCr)水平从0.5毫克/分升逐渐升高至1.62毫克/分升,直至1月份伴有蛋白尿和血尿。 2011年。肾脏活检显示,在光学显微镜下,肾小球基底膜(GBM)呈整体硬化(14%),纤维细胞新月形(28%)和瑞士干酪样外观。 IgG4染色阴性。免疫荧光检查显示沿GBM颗粒有IgG和C3的颗粒染色。诊断为MPO-ANCA相关性肾小球肾炎合并膜性肾病(MN)。由于肾脏活检标本中的慢性变化相对明显,而没有肾功能的急性增强,因此未进行免疫抑制治疗。此后,发生快速进行性肾功能不全(2011年5月,sCr,3.67 mg / dl),伴蛋白尿(〜2 g /天),血尿和血清MPO-ANCA水平升高。因此,于2011年5月进行了第二次肾脏活检,在光学显微镜下显示为全身硬化(42%)和细胞新月形(35%)。电子显微镜检查表明在GBM和肾小球系膜病变中有电子致密的沉积物。随后开始类固醇治疗,患者的肾功能得到部分改善。 MPO-ANCA水平降至正常范围内,血尿消失。 MPO-ANCA相关的肾小球肾炎合并MN是一种罕见的双肾小球病。但是,尿蛋白大量出现时应考虑并发症。怀疑继发性MN是由于缺乏IgG4染色和电子致密沉积物向系膜病变的分布。逐步出现的肾功能不全可能归因于MPO-ANCA相关性肾小球肾炎的间歇性增强。

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