Cardiovascular disease is a leading cause of death in survivors of immune-mediated thrombotic thrombocytopenic purpura (iTTP), but the epidemiology of major adverse cardiovascular events (MACE) in iTTP survivors is unknown. We evaluated the prevalence and risk factors for MACE, defined as the composite of non-fatal or fatal myocardial infarction (MI), stroke, and cardiac revascularization, during clinical remission in two large iTTP cohorts (Johns Hopkins University and Ohio State University). Of 181 patients followed for ≥ 3 months after recovery from acute iTTP, 28.6% had a MACE event over a median follow up of 7.6 years. Stroke was the most common type of MACE (18.2%), followed by non-fatal MI (6.6%), cardiac revascularization (4.9%) and fatal MI (0.6%). Compared to the general United States population, iTTP survivors were younger at first stroke in remission (males [56.5 years vs. 68.6 years, p = 0.031], females [49.7 years vs. 72.9 years, p < 0.001]) or MI in remission (males [56.5 years vs. 65.6 years, p < 0.001] and females [53.1 years vs. 72.0 years, p < 0.001]). Age (HR 1.03 [95% CI 1.002–1.054]), race (Black/Other vs. White) (HR 2.32 [95% CI 1.12–4.82]), and diabetes mellitus (HR 2.37 [95% CI 1.09–0.03]) were associated with MACE in a Cox regression model also adjusted for sex, hypertension, obesity, hyperlipidemia, chronic kidney disease, atrial fibrillation, autoimmune disease, and relapsing iTTP. Remission ADAMTS13 activity was not significantly associated with MACE. In conclusion, iTTP survivors experience high rates of MACE and may benefit from aggressively screening for and managing cardiovascular risk factors.

中文翻译：

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免疫介导的血栓性血小板减少性紫癜幸存者的主要不良心血管事件

心血管疾病是免疫介导的血栓性血小板减少性紫癜 (iTTP) 幸存者死亡的主要原因，但 iTTP 幸存者中主要不良心血管事件 (MACE) 的流行病学尚不清楚。我们在两个大型 iTTP 队列（约翰霍普金斯大学和俄亥俄州立大学）的临床缓解期间评估了 MACE 的患病率和危险因素，MACE 定义为非致命性或致命性心肌梗死 (MI)、中风和心脏血运重建的复合。在从急性 iTTP 恢复后随访 ≥ 3 个月的 181 名患者中，28.6% 的患者在中位随访 7.6 年期间发生了 MACE 事件。中风是最常见的 MACE 类型 (18.2%)，其次是非致命性 MI (6.6%)、心脏血运重建 (4.9%) 和致命性 MI (0.6%)。与美国总人口相比，p  = 0.031]，女性 [49.7 岁对 72.9 岁，p  < 0.001]）或 MI 缓解（男性 [56.5 岁对 65.6 岁，p  < 0.001] 和女性 [53.1 岁对 72.0 岁，p  < 0.001 ]). 年龄（HR 1.03 [95% CI 1.002–1.054]）、种族（黑人/其他人与白人）（HR 2.32 [95% CI 1.12–4.82]）和糖尿病（HR 2.37 [95% CI 1.09–0.03] ) 在 Cox 回归模型中与 MACE 相关，该模型还针对性别、高血压、肥胖、高脂血症、慢性肾病、心房颤动、自身免疫性疾病和复发性 iTTP 进行了调整。缓解期 ADAMTS13 活性与 MACE 无显着相关性。总之，iTTP 幸存者的 MACE 发生率很高，并且可能受益于积极筛查和管理心血管危险因素。