Chan R J, Goodman T A, Aretz T H, Lie J T
Massachusetts General Hospital, Harvard Medical School, Boston 02114, USA.
Arthritis Rheum. 1998 May;41(5):935-8. doi: 10.1002/1529-0131(199805)41:5<935::AID-ART22>3.0.CO;2-N.
Segmental mediolytic arteriopathy, a rare, noninflammatory arterial disease, is fundamentally a variant of fibromuscular dysplasia. The characteristic angiographic findings of segmental mediolytic arteriopathy include the "string of beads" and microaneurysms which are indistinguishable from those of vasculitis, and the correct diagnosis can be made only after histopathologic evaluation of the arterial lesions. Thrombosis, arterial wall hemorrhage, and dissection are among the complications of segmental mediolytic arteriopathy. We describe herein a patient with segmental mediolytic arteriopathy who presented with hemoperitoneum. The patient underwent urgent surgical repair of a ruptured hepatic artery aneurysm. The postoperative visceral arteriography findings led to a clinical diagnosis of polyarteritis nodosa, and immunosuppressive therapy was initiated. This treatment was stopped as soon as the correct biopsy diagnosis of segmental mediolytic arteriopathy was obtained through outside consultation. The patient recovered without drug treatment and was spared the potentially life-threatening complications of immunosuppression.
节段性中层溶解动脉病是一种罕见的非炎症性动脉疾病，本质上是纤维肌发育不良的一种变体。节段性中层溶解动脉病的典型血管造影表现包括“串珠样”改变和微动脉瘤，这些表现与血管炎难以区分，只有在对动脉病变进行组织病理学评估后才能做出正确诊断。血栓形成、动脉壁出血和夹层分离是节段性中层溶解动脉病的并发症。我们在此描述一名患有节段性中层溶解动脉病并出现腹腔积血的患者。该患者接受了破裂肝动脉瘤的紧急手术修复。术后内脏动脉造影结果导致临床诊断为结节性多动脉炎，并开始了免疫抑制治疗。一旦通过外部会诊获得节段性中层溶解动脉病的正确活检诊断，该治疗即停止。患者未经药物治疗而康复，避免了免疫抑制可能危及生命的并发症。
