Large vessel disease as a potentially treatable cause of devastating critical digital ischaemia in systemic sclerosis
Large vessel disease as a potentially treatable cause of devastating critical digital ischaemia in systemic sclerosis
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大血管疾病是系统性硬化症中破坏性严重指缺血的潜在可治疗原因
DOI：
10.1007/s10067-020-05128-x
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发表时间：
2020
影响因子：
3.4
通讯作者：
R. Kilding
中科院分区：
文献类型：
--
作者：
A. Haque;T. Cleveland;L. Powell;S. Stephenson;M. Hughes;R. Kilding
一位60岁的女性，已知皮肤SSc有限（抗着丝粒抗体阳性），有严重的指血管疾病史，表现为进行性指血管缺血。她的病史包括双侧（动脉周围）交感神经切除术和手指截肢。她没有可改变的心血管危险因素，正在服用西地那非治疗ssc -手指血管病变。发病时，左侧食指和拇指指尖出现溃疡，并伴有进行性坏死(a)。1）. 她有明显的疼痛，接受了5天的静脉注射伊洛前列素。尽管如此，她的左手腕和前臂出现了非常轻微的肿胀，桡动脉和肱动脉脉搏缺失。怀疑血流受损的更近端原因，因此，紧急要求动脉双工扫描显示桡动脉闭塞，尺动脉血流逆转。肱动脉的血流严重受阻。因此，采用计算机断层血管造影（CTA）进行治疗干预。CTA显示左侧锁骨下动脉短暂闭塞(b)，导管血管造影(c)证实了这一点，在放置支架(d)之前进行了导管血管造影以成功缓解阻塞。随后，她的临床状况改善，疼痛减轻，缺血组织损失受限。SSc的严重指端缺血是一种医疗紧急情况，因为早期识别近端（大）血管疾病可能有助于挽救组织的血运重建[1-3]。有报道称SSc发生大血管疾病的风险增加，特别是那些疾病有限且抗着丝粒抗体阳性的患者（如本例患者）[1-3]。其他潜在的原因包括（但不限于）炎症（如血管炎）、感染和栓塞。临床医生必须保持高度的怀疑指数，并始终积极考虑大血管疾病的ssc相关的临界数字缺血。
A 60-year-old lady with known limited cutaneous SSc (anti-centromere antibody positive) and a history of severe digital vascular disease presented with progressive digital vascular ischaemia. Her history included bilateral (periarterial) sympathectomy and digital amputation. She had no modifiable cardiovascular risk factors and was taking sildenafil for SSc-digital vasculopathy. At presentation, there was ulceration of tip of the left index finger and thumb, with progressive necrosis (a)(Fig. 1). She was in significant pain and received a 5-day course of intravenous iloprost. Despite this, she developed very tender swelling of her left wrist and forearm with absent radial and brachial pulses. A more proximal cause for compromised flow was suspected, and therefore, an urgently requested arterial Duplex scan demonstrated an occluded radial artery, with reversed flow in the ulnar artery. Flow in the brachial artery was noted to be severely damped. Therefore, computerised tomography angiography (CTA) was performed with a view to therapeutic intervention.CTA demonstrated a short occlusion of the left subclavian artery (b), which was confirmed by a catheter angiogram (c), performed prior to placement of a stent (d) to successfully relieve the obstruction. Subsequently, her clinical condition improved with reduced pain and limitation of ischaemic tissue loss. Critical digital ischaemia in SSc is a medical emergency because early recognition of proximal (large) vessel disease can potentially be amenable to tissue-saving revascularisation [1–3]. An increased risk of macrovascular disease has been reported in SSc, in particular, in those with limited disease and positive anti-centromere antibodies (like our patient)[1–3]. Other potential causes include (but are not limited to) inflammation (eg vasculitis), infection and embolism [3]. Clinicians must maintain a high index of suspicion and always actively consider large vessel disease in SSc-related critical digital ischaemia.