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首页> 外文期刊>Clinical and experimental nephrology >Thrombotic thrombocytopenic purpura in a patient with rapidly progressive glomerulonephritis with both anti-glomerular basement membrane antibodies and myeloperoxidase anti-neutrophil cytoplasmic antibodies.
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Thrombotic thrombocytopenic purpura in a patient with rapidly progressive glomerulonephritis with both anti-glomerular basement membrane antibodies and myeloperoxidase anti-neutrophil cytoplasmic antibodies.

机译：快速抗肾小球肾炎合并抗肾小球基底膜抗体和髓过氧化物酶抗中性粒细胞胞浆抗体的患者的血栓性血小板减少性紫癜。

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摘要

A 61-year-old Japanese woman with rapidly progressive glomerulonephritis exhibited both anti-glomerular basement membrane (GBM) antibodies (920 EU) and myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA; 66 EU). Multiple plasma exchanges with fresh frozen plasma preceded by 500 mg/day intravenous methylprednisolone and 30 mg/day oral prednisolone decreased anti-GBM antibody and MPO-ANCA antibody titers to 106 EU and below 10 EU (normal ranges), respectively. Thrombotic thrombocytopenic purpura (TTP) manifests itself as a moderate decrease in the activity of disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13 (ADAMTS13) protein levels to 35% of normal; ADAMTS13 deficiency is only symptomatic when levels are less than 50%. This patient's disease was resistant to extensive plasma exchange, leading to her death from respiratory distress and perforation of the alimentary tract secondary to cytomegalovirus infection. Autopsy demonstrated severe crescentic glomerulonephritis associated with linear IgG, IgA, IgM, and C3 deposits along the glomerular capillary walls. This case is an uncommon example of combined double antibody-positive crescentic glomerulonephritis and refractory TTP.
机译：一名61岁的日本女性患有快速进行性肾小球肾炎，表现出抗肾小球基底膜（GBM）抗体（920 EU）和髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA; 66 EU）。与新鲜冷冻血浆进行多次血浆交换后，每天静脉给予500 mg泼尼松龙和口服泼尼松龙30 mg /天，抗GBM抗体和MPO-ANCA抗体的滴度分别降至106 EU和10 EU以下（正常范围）。血栓性血小板减少性紫癜（TTP）表现为具有血小板反应蛋白1型基序13（ADAMTS13）的Disintegrin-like和金属蛋白酶的活性适度降低至正常水平的35％;仅当水平低于50％时才有ADAMTS13缺乏症的症状。该患者的疾病抵抗广泛的血浆交换，导致其因呼吸窘迫而死亡，并继发巨细胞病毒感染导致消化道穿孔。尸检显示严重新月形肾小球肾炎，与沿肾小球毛细血管壁的线性IgG，IgA，IgM和C3沉积有关。这种情况是双抗体阳性新月形肾小球肾炎和难治性TTP合并症的罕见例子。

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