The Fourth Clinical Medical College of Guangzhou University of Chinese Medicine, No.1, Fuhua Road, Futian District, Shenzhen, Guangdong, 518033, China.
The Department of Rheumatology, Shenzhen Traditional Chinese Medicine Hospital, No.1, Fuhua Road, Futian District, Shenzhen, Guangdong, 518033, China.
Clin Rheumatol. 2022 Oct;41(10):3227-3235. doi: 10.1007/s10067-022-06163-6. Epub 2022 Jun 11.
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.
显微镜下多血管炎（MPA）是一种与抗中性粒细胞胞浆抗体（ANCA）相关的系统性小血管血管炎，主要导致肾脏和肺部损伤。蛛网膜下腔出血（SAH）是一种危及生命的中枢神经系统（CNS）表现，在 ANCA 相关性血管炎（AAV）患者中很少发生。我们报告了一例年轻男性自发性 SAH 复发和活动性肾炎的病例。该患者接受了糖皮质激素脉冲和静脉环磷酰胺（CTX）联合治疗，同时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 34 例先前报道的伴有 AAV 的 SAH 患者的临床特征，其中包括 6 例 MPA、8 例肉芽肿性多血管炎（GPA）和 19 例嗜酸性肉芽肿性多血管炎（EGPA），以及 1 例未分类的 AAV。所有病例均表现为活动性血管炎的特征。并发肾炎和周围神经病分别见于 MPA 和 EGPA 合并 SAH 的病例。在 GPA 和 SAH 患者中，肾脏和肺部表现更为突出。10 例患者存在动脉瘤异常，6 例患者存在心脏异常。31 例患者接受了糖皮质激素治疗，18 例患者接受了联合免疫抑制剂治疗。SAH 患者的死亡率为 38.2%。AAV 和 SAH 患者中存在脑血管事件或心脏受累与死亡率增加 64.3%相关。我们的研究表明，SAH 应引起患有 AAV 的患者的警惕。早期诊断并积极进行免疫抑制治疗有助于改善 SAH 患者的预后。
