Taylor Marian H, McFadden John A, Bolster Marcy B, Silver Richard M
Department of Medicine, Medical University of South Carolina, Charleston 29425, USA.
J Rheumatol. 2002 Jan;29(1):102-6.
Microvascular disease is one of the hallmarks of systemic sclerosis (SSc, scleroderma), but macrovascular involvement also exists in some patients. Patients with SSc may have severe Raynaud's phenomenon (RP) characterized by refractory digital ulcerations. We investigated if large artery involvement, that is, ulnar artery occlusion, has a role in the development of refractory digital ulcerations, and if both screening for this involvement and revascularization of the ulnar artery occlusive disease may improve digital ulcer healing.
A retrospective chart review was performed of 15 patients with SSc, all of whom had severe RP and digital ulceration, together with a positive Allen test and ulnar artery occlusive disease documented by angiography.
Women outnumbered men 2:1, with limited disease predominating (7), 5 patients having diffuse cutaneous disease and 3 overlap syndromes. All patients had positive antinuclear antibody and capillary microscopy findings consistent with SSc. Antiphospholipid antibodies were present in 4 of 6 patients tested. Tobacco use was seen in 5 patients, only 2 of whom were current smokers. All patients failed conventional medical therapy (nitrates, calcium channel blockers, antiplatelet agents) for RP and digital ulceration. Only 1/8 patients improved with stellate ganglion block, and one patient had no improvement following digital sympathectomy. Eight patients underwent ulnar artery revascularization combined with digital sympathectomy, and 8 experienced dramatic improvement in RP and healing of digital ulcers.
An Allen test should be performed routinely on all SSc patients with severe RP and refractory digital ulceration to investigate the possibility of ulnar artery occlusive disease. If suspected ulnar artery occlusion is confirmed by angiography or ultrasonography, ulnar artery revascularization with or without digital sympathectomy should be considered in patients who fail conventional medical therapy.
微血管疾病是系统性硬化症（SSc，硬皮病）的标志性特征之一，但部分患者也存在大血管受累情况。SSc患者可能出现以难治性指端溃疡为特征的严重雷诺现象（RP）。我们研究了大动脉受累，即尺动脉闭塞，在难治性指端溃疡发生过程中是否起作用，以及对这种受累情况的筛查和尺动脉闭塞性疾病的血运重建是否能促进指端溃疡愈合。
对15例SSc患者进行回顾性病历审查，所有患者均有严重RP和指端溃疡，同时艾伦试验阳性且血管造影证实存在尺动脉闭塞性疾病。
女性与男性比例为2:1，以局限性疾病为主（7例），5例为弥漫性皮肤疾病，3例为重叠综合征。所有患者抗核抗体阳性，毛细血管显微镜检查结果与SSc相符。6例接受检测的患者中有4例存在抗磷脂抗体。5例患者有吸烟史，其中仅2例为现吸烟者。所有患者针对RP和指端溃疡的传统药物治疗（硝酸盐、钙通道阻滞剂、抗血小板药物）均无效。星状神经节阻滞仅使1/8的患者病情改善，1例患者行指交感神经切除术后无改善。8例患者接受了尺动脉血运重建联合指交感神经切除术，8例患者的RP显著改善，指端溃疡愈合。
对于所有患有严重RP和难治性指端溃疡的SSc患者，应常规进行艾伦试验，以排查尺动脉闭塞性疾病的可能性。如果血管造影或超声检查证实怀疑的尺动脉闭塞，对于传统药物治疗无效的患者，应考虑进行尺动脉血运重建，可联合或不联合指交感神经切除术。
