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Abstract：
The coexistence of anti-glomerular basement membrane (anti-GBM) disease with thrombotic microangiopathy (TMA) is rarely encountered, and the clinical characteristics of this phenomenon are not well known.A 76-year-old Japanese woman with a history of idiopathic pulmonary disease was diagnosed with anti-GBM disease due to rapidly progressive glomerulonephritis and a positive anti-GBM antibody test result. We treated the patient with hemodialysis, glucocorticoids, and plasmapheresis. During treatment, the patient suddenly became comatose. TMA was then diagnosed because of thrombocytopenia and microangiopathic hemolytic anemia. The activity of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) was retained at 48%. Although we continued the treatment, the patient died of respiratory failure. An autopsy revealed the cause of respiratory failure to be an acute exacerbation of interstitial pneumonia. The clinical findings of the renal specimen indicated anti-GBM disease; however, there were no lesions suggestive of TMA. A genetic test did not reveal an apparent genetic mutation of the atypical hemolytic uremic syndrome.We conducted a literature review of past case reports of anti-GBM disease with TMA. The following clinical characteristics were obtained. First, 75% of the cases were reported in Asia. Second, TMA tended to appear during the treatment course for anti-GBM disease and usually resolved within 12 weeks. Third, ADAMTS-13 activity was retained above 10% in 90% of the cases. Fourth, central nervous system manifestations occurred in more than half of the patients. Fifth, the renal outcome was very poor. Further studies are required to understand the pathophysiology of this phenomenon.
摘要：
很少遇到抗肾小球基底膜（抗GBM）疾病与血栓性微血管病（TMA）的共存，这种现象的临床特征尚不清楚。一名有特发性肺病史的76岁日本妇女因快速进展性肾小球肾炎和抗GBM抗体测试结果阳性而被诊断患有抗GBM疾病。我们用血液透析治疗病人,糖皮质激素,和血浆置换.治疗期间,病人突然昏迷了。然后由于血小板减少症和微血管病性溶血性贫血而诊断为TMA。具有血小板反应蛋白1型基序13（ADAMTS-13）的崩解素样和金属蛋白酶的活性保持在48％。虽然我们继续治疗,患者死于呼吸衰竭.尸检显示呼吸衰竭的原因是间质性肺炎的急性加重。肾脏标本的临床表现提示抗GBM疾病；然而，没有病变提示TMA。基因测试未发现非典型溶血性尿毒综合征的明显基因突变。我们对过去的TMA抗GBM疾病病例报告进行了文献回顾。获得以下临床特征。首先,75%的病例报告发生在亚洲。第二,TMA倾向于在抗GBM疾病的治疗过程中出现，通常在12周内消退。第三，在90%的病例中,ADAMTS-13活性保持在10%以上。第四，超过一半的患者出现中枢神经系统表现。第五,肾脏结局很差.需要进一步的研究来了解这种现象的病理生理学。
