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首页> 外文期刊>CEN Case Reports >AP-VAS 2012 case report: a case of systemic MPO-ANCA-associated vasculitis that demonstrated brain infarction and immunohistochemically MPO-positive capillaries
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AP-VAS 2012 case report: a case of systemic MPO-ANCA-associated vasculitis that demonstrated brain infarction and immunohistochemically MPO-positive capillaries

机译：AP-VAS 2012病例报告：一例系统性MPO-ANCA相关性血管炎，表现出脑梗塞和免疫组化MPO阳性毛细血管

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We present a case of an aged male with myeloperoxidase anti-neutrophil cytoplasmic autoantibody (MPO-ANCA)-associated vasculitis with onset of brain infarction that demonstrated immunohistochemically MPO-positive capillaries at autopsy. The patient initially presented with gait difficulty and right-sided weakness. Since an imaging study revealed brain infarction, he was admitted to our hospital and medicated by antiplatelet agents. Continuous fever and elevated serum C-reactive protein (CRP), hematuria of glomerular origin, renal dysfunction, and high serum titer of MPO-ANCA were detected. Systemic toxicoderma appeared, and skin biopsy revealed small-vessel vasculitis; thus, he was diagnosed with MPO-ANCA-associated vasculitis. Steroid therapy (methylprednisolone 30 mg/day) was started, and general status improved. However, he died of shock 6 days after the start of the therapy. Autopsy revealed massive retroperitoneal hemorrhage with necrotizing small-vessel vasculitis in systemic organs including retroperitoneum, skin, brain, testes, and kidneys. Immunohistochemically, infiltration of MPO-positive white blood cells into the capillaries was occasionally observed, along with the features of MPO-positive capillaries. Cerebrovascular involvement of MPO-ANCA-associated vasculitis is rare compared with renal and pulmonary manifestations, having been reported to occur in up to 4 % of patients. Furthermore, as we have recently reported, MPO-immunopositive capillaries may appear only during the hyperacute stage of the disease. Therefore, the present case represents the unique combination of these two rare manifestations.
机译：我们介绍了一名老年男性，患有髓过氧化物酶抗中性粒细胞胞质自身抗体（MPO-ANCA）相关的血管炎伴脑梗塞，在尸检中显示免疫组织化学MPO阳性毛细血管。患者最初表现为步态困难和右侧无力。由于影像学检查显示脑梗塞，他被送入我们医院并接受抗血小板药物治疗。检测到持续发烧和血清C反应蛋白（CRP）升高，肾小球血尿，肾功能不全以及MPO-ANCA的高血清滴度。出现全身性毒性皮肤病，皮肤活检发现小血管血管炎；因此，他被诊断出患有MPO-ANCA相关的血管炎。开始类固醇治疗（甲基泼尼松龙30毫克/天），总体状况得到改善。但是，他在治疗开始后的6天死于休克。尸检显示腹膜后大量出血，包括腹膜后，皮肤，脑，睾丸和肾脏在内的全身器官坏死性小血管血管炎。在免疫组织化学上，偶尔观察到MPO阳性白细胞渗入毛细血管，以及MPO阳性毛细血管的特征。与肾和肺部表现相比，MPO-ANCA相关血管炎的脑血管受累很少见，据报道发生在多达4％的患者中。此外，正如我们最近报道的那样，MPO免疫阳性毛细血管可能仅在疾病的超急性期出现。因此，本案代表了这两种罕见表现的独特组合。

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