Cohen Debbie L, Soulen Michael C
Renal, Electrolyte and Hypertension Division, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; and
Division of Interventional Radiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Clin J Am Soc Nephrol. 2015 Apr 7;10(4):696-702. doi: 10.2215/CJN.10171014. Epub 2015 Jan 12.
This case presented challenging diagnostic and management issues in a young healthy man who presented with abdominal pain and new-onset hypertension. The differential diagnosis evolved over the course of the clinical presentation. The patient had severe vascular involvement of his renal and basal cerebral arteries that initially was assumed to be due to a vasculitic process or hypercoagulable state. Finally it became apparent that the patient did not have a systemic illness but rather a localized vascular disease most likely due to segmental arterial mediolysis, a rare, under-recognized condition that can potentially be fatal. This condition is often difficult to distinguish from fibromuscular dysplasia. It is important to recognize and correctly diagnose the condition, particularly in the acute phase of the disease, because delay in diagnosis can contribute to morbidity and mortality.
该病例为一名年轻健康男性,出现腹痛和新发高血压,带来了具有挑战性的诊断和管理问题。鉴别诊断在临床过程中不断演变。患者的肾动脉和基底脑动脉有严重血管受累,最初认为是血管炎过程或高凝状态所致。最终发现患者并无全身性疾病,而是患有一种局部血管疾病,很可能是节段性动脉中层溶解,这是一种罕见且未得到充分认识的疾病,可能会致命。这种疾病常难以与纤维肌发育异常相区分。认识并正确诊断该疾病很重要,尤其是在疾病急性期,因为诊断延迟会导致发病率和死亡率上升。