Perruquet J L, Davis D E, Harrington T M
Arch Intern Med. 1986 Feb;146(2):289-91.
Five elderly patients with aortic arch syndrome secondary to large-vessel vasculitis all presented with upper-extremity claudication and absence of blood pressure and pulses in the affected extremity. Diagnosis was suspected by a markedly elevated erythrocyte sedimentation rate associated with constitutional symptoms and angiograms showing changes consistent with arteritis of the aortic arch vessels. Treatment with high-dose corticosteroids resulted in rapid resolution of constitutional symptoms and improved functional use of the upper extremity. Recognition of this steroid-responsive disease is essential to avoid unnecessary reconstructive surgery and to prevent catastrophic events such as aortic dissection or visual loss, which may result from the underlying disease process.
5例继发于大血管血管炎的主动脉弓综合征老年患者均表现为上肢间歇性跛行,患侧上肢无血压及脉搏。红细胞沉降率显著升高并伴有全身症状,血管造影显示与主动脉弓血管动脉炎相符的改变,据此怀疑为该病。高剂量皮质类固醇治疗使全身症状迅速缓解,上肢功能使用得到改善。认识这种对类固醇有反应的疾病对于避免不必要的重建手术以及预防诸如主动脉夹层或视力丧失等可能由潜在疾病过程导致的灾难性事件至关重要。