Spontaneous subarachnoid hemorrhage (SAH) occurs due to intracranial aneurysm rupture in most cases. Rheumatic disease may cause vessel wall inflammation, which can increase the risk of rupture. However, the characteristics of SAH with rheumatic disease are unknown. This study aimed to evaluate SAH features in patients with rheumatic disease. We retrospectively analyzed clinical data of 5066 patients from the Nagasaki SAH Registry Study who had been diagnosed with aneurysmal SAH between 2001 and 2018. We evaluated the SAH characteristics in patients with rheumatic disease using multivariable logistic regression analysis. In total, 102 patients (2.0%, 11 men and 91 women, median age 69.0 [57.0–75.5]) had rheumatic disease. In these patients, univariate logistic regression analysis showed that sex, hypertension, family history of SAH, smoking history, World Federation of Neurosurgical Societies grade on admission, aneurysm size, multiple aneurysms, treatment, and symptomatic spasms were associated with SAH. Multivariable logistic regression analysis showed that characteristics independently associated with SAH in rheumatic disease were female sex (odds ratio [OR] 3.38; 95% confidence interval [CI] 1.81–6.93, P < 0.001), hypertension (OR 0.60; 95% CI 0.40–0.90, P = 0.012), family history of SAH (OR 0.18; 95% CI 0.01–0.80, P = 0.020), small ruptured aneurysms (OR 1.50; 95% CI 1.02–2.24, P = 0.048), and multiple aneurysms (OR 1.69; 95% CI 1.09–2.58, P = 0.021) in comparison with SAH without rheumatic disease. In conclusion, SAH in patients with rheumatic disease was characterized by small multiple aneurysms, regardless of the low incidence of hypertension and family history of SAH.

在大多数情况下，自发性蛛网膜下腔出血（SAH）是由于颅内动脉瘤破裂引起的。风湿性疾病可能会导致血管壁发炎，这会增加破裂的风险。然而，SAH伴风湿性疾病的特征尚不清楚。本研究旨在评估风湿病患者的 SAH 特征。我们回顾性分析了长崎 SAH 登记研究中 2001 年至 2018 年间被诊断为动脉瘤性 SAH 的 5066 名患者的临床数据。我们使用多变量逻辑回归分析评估了风湿病患者的 SAH 特征。总共有 102 名患者（2.0%，11 名男性和 91 名女性，中位年龄 69.0 [57.0-75.5]）患有风湿性疾病。在这些患者中，单因素logistic回归分析显示性别、高血压、SAH家族史、吸烟史、入院时世界神经外科学会联合会分级、动脉瘤大小、多发性动脉瘤、治疗和症状性痉挛与 SAH 相关。多变量逻辑回归分析显示，与风湿病 SAH 独立相关的特征是女性（优势比 [OR] 3.38；95% 置信区间 [CI] 1.81-6.93，P  < 0.001）、高血压（OR 0.60；95% CI 0.40–0.90，P  = 0.012）、SAH 家族史（OR 0.18；95% CI 0.01–0.80，P  = 0.020）、小动脉瘤破裂（OR 95550） % CI 1.02–2.24, P  = 0.048) 和多发性动脉瘤 (OR 1.69; 95% CI 1.09–2.58, P  = 0.021) 与无风湿性疾病的 SAH 相比。总之，风湿性疾病患者的 SAH 以小的多发性动​​脉瘤为特征，而不管高血压的低发病率和 SAH 家族史。