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AP-VAS 2012 case report: anti-glomerular basement membrane disease with high titer of myeloperoxidase anti-neutrophil cytoplasmic antibody—an autopsy case report

机译:AP-VAS 2012病例报告:滴度高的髓过氧化物酶抗中性粒细胞胞浆抗体抗肾小球基底膜病-尸检病例报告

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It has been reported that patients who are positive for both myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement membrane (GBM) antibody have a poor prognosis. We present an autopsy case of anti-GBM disease with a high titer of MPO-ANCA. The patient was a 77-year-old woman with a medical history of idiopathic interstitial pneumonia. After being treated for bacterial pneumonia, she was referred to our hospital for evaluation of non-nephrotic range proteinuria, hematuria, and a course of rapidly progressive glomerulonephritis. Results of urinalysis were 2+ for protein and 3+ for blood, with many dysmorphic red blood cells observed in the urinary sediment. A sample of a 24-h urine collection contained 0.3 g protein. The serum creatinine concentration was 5.0 mg/dl on admission. The patient tested positive for MPO-ANCA at a titer of >640 EU and for anti-GBM antibody at a titer of 14 EU. Renal biopsy revealed glomerulonephritis with crescent formation, and immunofluorescence studies showed that the glomeruli had a generalized linear fluorescence and anti-immunoglobulin G (IgG) and C3 along the peripheral glomerular capillaries. She was diagnosed with anti-GBM disease. Treatment was started with intravenous prednisolone and oral cyclophosphamide, followed by plasma exchange. Despite improved renal function, she died of pulmonary hemorrhage. Autopsy revealed deposits of IgG and C3 in the basement membranes of lung alveoli.
机译:据报道,髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)和抗肾小球基底膜(GBM)抗体均为阳性的患者预后较差。我们提出了具有高滴度MPO-ANCA的抗GBM疾病尸检病例。该患者是一名77岁女性,有特发性间质性肺炎病史。在接受细菌性肺炎治疗后,她被转诊到我院,以评估非肾病性蛋白尿,血尿和一个快速进展的肾小球肾炎的病程。尿液分析的结果是蛋白质的2+和血液的3+,在尿沉渣中观察到许多畸形的红细胞。 24小时尿液样本中包含0.3 g的蛋白质。入院时血清肌酐浓度为5.0 mg / dl。该患者的MPO-ANCA滴度> 640 EU呈阳性,抗GBM抗体的滴度14 EU呈阳性。肾活检显示肾小球肾炎具有新月形形成,免疫荧光研究表明,肾小球沿外周肾小球毛细血管具有广义的线性荧光和抗免疫球蛋白G(IgG)和C3。她被诊断出患有抗GBM疾病。用静脉泼尼松龙和口服环磷酰胺开始治疗,然后进行血浆置换。尽管肾功能得到改善,但她死于肺出血。尸检显示IgG和C3沉积在肺泡基底膜中。

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