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AP-VAS 2012 case report: a case of lupus nephritis with predominant synchronous cellular crescent formation and myeloperoxidase-antineutrophil cytoplasmic antibody seropositivity

机译:AP-VAS 2012病例报告:一例主要发生同步细胞新月形成且髓过氧化物酶-抗中性粒细胞胞浆抗体血清阳性的狼疮性肾炎

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Several cases with an overlap of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) and lupus nephritis (LN) features have been reported in recent years. However, the clinical and the pathologic features of this condition, including mode of development, histology, and response to treatment, are not fully understood. We report a 77-year-old woman who was diagnosed with Sjögren syndrome 15 years previously. The patient presented with acute worsening of renal function and was diagnosed with new-onset systemic lupus erythematosus. A renal biopsy specimen revealed proliferative LN with synchronous cellular crescents. She was also seropositive for myeloperoxidase-ANCA. Together with the positive staining for immunoglobulins and complement factors on immunofluorescence microscopy and scant subendothelial deposits by electron microscopy, we reached a diagnosis of ANCA-associated crescentic GN overlapping with LN. Although immunosuppressive treatment with methylprednisolone pulse therapy and intravenous cyclophosphamide followed by oral predonisolone was initiated, along with intermittent hemodialysis, these treatments did not induce remission of her GN. Therefore, she continued regular intermittent hemodialysis. However, she died because of candida pneumonia 4 months after admission. Generally, the glomeruli of patients with ANCA-associated GN exhibit different stages of crescents, namely cellular, fibrocellular, or fibrous. The histologically synchronous crescents in this case indicate that ANCA-associated GN overlapping with LN can progress more rapidly than that without LN. This overlapping type of GN may be resistant to conventional immunosuppressive therapies.
机译:近年来,已有几例抗中性粒细胞胞浆抗体(ANCA)相关的肾小球肾炎(GN)和狼疮性肾炎(LN)特征重叠的病例。但是,这种病的临床和病理特征,包括发展方式,组织学和对治疗的反应,还没有被完全理解。我们报告了一位15岁以前被诊断患有Sjögren综合征的77岁女性。该患者表现为肾功能急性恶化,并被诊断为新发系统性红斑狼疮。肾活检标本显示增生性LN伴同步细胞新月。她的髓过氧化物酶-ANCA也呈血清阳性。加上免疫荧光显微镜检查和免疫球蛋白和补体因子的阳性染色,以及电子显微镜检查发现的内皮下沉积物少,我们诊断出与LN重叠的ANCA相关新月形GN。尽管开始使用甲基泼尼松龙脉冲疗法,静脉内环磷酰胺和口服泼尼松龙的免疫抑制治疗,以及间歇性血液透析,但这些治疗并未诱导其GN缓解。因此,她继续定期进行间歇性血液透析。然而,她在入院后4个月死于念珠菌性肺炎。通常,ANCA相关性GN患者的肾小球表现出新月的不同阶段,即细胞,纤维细胞或纤维状。在这种情况下,组织学上同步的新月表明,与LN重叠的ANCA相关GN可以比没有LN的GN更快地进行。 GN的这种重叠类型可能对常规的免疫抑制疗法有抵抗力。

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