Differentiating between segmental arterial mediolysis and other arterial vasculopathies to establish an early diagnosis – a systematic literature review and proposal of new diagnostic criteria

Segmental arterial mediolysis (SAM) is a rare vascular disease, characterized by acute but transient vulnerability of the wall of medium-sized arteries. The most characteristic feature of SAM is its biphasic course: an injurious phase marked by acute weakness of the arterial wall leading to acute dissection and/or hemorrhage, followed by a reparative phase in which granulation tissue and fibrosis restore the injured arterial wall. Residual stenosis, aneurysms, and/or arterial wall irregularities may remain visible on future imaging studies. Differentiating between SAM and other arterial vasculopathies is difficult due to its similarities with many other vascular diseases, such as vasculitis, fibromuscular dysplasia, inherited connective tissue disorders, and isolated visceral artery dissection. In this systematic review, we provide an overview on SAM, with an emphasis on the differential diagnosis and diagnostic work-up. We propose new diagnostic criteria to help establish a prompt diagnosis of SAM, illustrated by case examples from our multidisciplinary vascular clinic.

节段性动脉中层溶解症（Segmental arterial mediolysis, SAM）是一种罕见的血管疾病，以中等大小动脉壁的急性但一过性脆弱为特征。该病最具特征性的表现为双相病程：损伤期以动脉壁急性薄弱为特点，可引发急性动脉夹层和/或出血；随后进入修复期，肉芽组织与纤维化可修复受损的动脉壁。后续影像学检查中，仍可观察到残留狭窄、动脉瘤及/或动脉壁不规则等后遗表现。由于SAM与诸多其他血管疾病存在相似性，如血管炎、纤维肌发育不良、遗传性结缔组织病以及孤立性内脏动脉夹层等，因此将其与其他动脉血管病变鉴别存在较大难度。本系统综述对SAM进行了全面概述，重点关注其鉴别诊断与诊断流程，并提出全新的诊断标准以助力SAM的快速确诊，同时辅以多学科血管门诊的病例实例加以说明。