Abstract：
Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) and predominantly causes kidney and pulmonary injuries. Subarachnoid hemorrhage, a life-threatening manifestation of the central nervous system (CNS), rarely occurs in patients with ANCA-associated vasculitis (AAV). We report the case of a young man with spontaneous SAH recurrence and active nephritis. The patient was treated with a glucocorticoid pulse and intravenous cyclophosphamide (CTX) in combination with decreasing cerebral perfusion pressure and analgesic therapy. All the patients\' symptoms except the proteinuria resolved. We reviewed the clinical characteristics of 34 previously reported cases of SAH with AAV, comprising six cases of MPA, eight cases of granulomatosis with polyangiitis (GPA), and 19 cases of eosinophilic granulomatosis with polyangiitis (EGPA), and one case of unclassified AAV. All the cases showed features of active vasculitis. Concomitant nephritis and peripheral neuropathy were found in the MPA and EGPA cases with SAH, respectively. Renal and pulmonary manifestations were predominant in the patients with GPA and SAH. Ten patients had aneurysmal abnormalities, and six patients had cardiac abnormalities. Thirty-one patients were treated with glucocorticoids, and 18 patients received concurrent immunosuppressants. Patients with SAH had a mortality rate of 38.2%. The presence of cerebrovascular events or cardiac involvement in patients with AAV and SAH is associated with increased mortality of 64.3%. Our study indicates that SAH should be cautioned as a disease occurring in patients with AAV. Early diagnosis with aggressive immunosuppressive therapy can help improve the prognosis of patients with SAH.
摘要：
显微镜性多血管炎（MPA）是一种与抗中性粒细胞胞浆抗体（ANCA）相关的全身性小血管炎，主要引起肾脏和肺部损伤。蛛网膜下腔出血,中枢神经系统（CNS）的威胁生命的表现，很少发生在ANCA相关性血管炎（AAV）患者中。我们报告了一例自发性SAH复发和活动性肾炎的年轻人。患者接受糖皮质激素脉冲和静脉环磷酰胺（CTX）联合降低脑灌注压和镇痛治疗。除蛋白尿外，所有患者的症状均得到缓解。我们回顾了先前报道的34例AAV合并SAH的临床特征，包括6例MPA，肉芽肿性多血管炎（GPA）8例，和19例嗜酸性肉芽肿性多血管炎（EGPA），和一例未分类AAV。所有病例均表现为活动性血管炎。在SAH的MPA和EGPA病例中发现并发肾炎和周围神经病变，分别。GPA和SAH患者以肾脏和肺部表现为主。十名患者有动脉瘤异常，6名患者有心脏异常。31例患者接受糖皮质激素治疗，18例患者同时接受免疫抑制剂治疗。SAH患者的死亡率为38.2%。AAV和SAH患者的脑血管事件或心脏受累的存在与64.3％的死亡率增加有关。我们的研究表明，SAH应被警告为AAV患者中发生的疾病。早期诊断采用积极的免疫抑制治疗有助于改善SAH患者的预后。
