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伴有周围缺血表现的原发性血管炎模式:病例系列报告及血管外科的作用

Pattern of primary vasculitis with peripheral ischemic manifestations: report of a case series and role of vascular surgery.

作者信息

Gheita Tamer A, Samad Hisham M Abdel, Mahdy Maher A, Kamel Alaa B

机构信息

Rheumatology Department, Faculty of Medicine, Cairo University, Egypt.

出版信息

Curr Rheumatol Rev. 2014;10(2):126-30. doi: 10.2174/1573397110666150120103559.

DOI:10.2174/1573397110666150120103559
PMID:25599681
Abstract

AIM OF THE WORK

The aim of the present work was to study the role of vascular surgery in the management of primary vasculitis patients with peripheral ischemic manifestations.

PATIENTS AND METHODS

Ten primary vasculitis patients with peripheral ischemic manifestations were studied and reviewed for the diagnosis, clinical manifestations, investigations, treatment options and role of vascular surgery. The Birmingham Vasculitis Activity Score (BVAS) was recorded.

RESULTS

Giant cell arteritis was present in one patient; granulomatosis with polyangiitis in 5, essential cryoglobulinemic vasculitis in 3 and 1 (child) had Henoch-Schönlein purpura. They showed the following peripheral vascular manifestations: intermittent claudications, Raynauds, deep venous thrombosis and thrombophelebitis in 10% each; digital ulceration and trophic changes in 20% while acrocyanosis and dry gangrene were present in 30%. Renal involvement was present in 60% of patients. The mean BVAS was 11.5 ± 6.57 at initial presentation. The disease activity remarkably improved over the disease course in all patients to be at their last visit (2.6 ± 2.22) (p=0.002). Regarding the vascular surgery role in their management, in addition to their medical treatment, 40% required an additional surgical intervention. Two had a minor amputation of the toes; one performed thoracoscopic cervical sympathectomy and another needed tibial angioplasty.

CONCLUSION

Primary vasculitis patients presenting with peripheral ischemic manifestations require surgical attention. Their management is essentially medical and individualized to the diagnosis and presenting symptoms. Endovascular treatment may offer a safe and less invasive approach in high surgical risk patients. Sympathectomy is of high therapeutic potential in those with severe pain and trophic changes.

摘要

工作目的

本研究旨在探讨血管外科手术在治疗伴有外周缺血表现的原发性血管炎患者中的作用。

患者与方法

对10例伴有外周缺血表现的原发性血管炎患者进行了研究,回顾了其诊断、临床表现、检查、治疗选择以及血管外科手术的作用。记录了伯明翰血管炎活动评分(BVAS)。

结果

1例患者为巨细胞动脉炎;5例为肉芽肿性多血管炎;3例为原发性冷球蛋白血症性血管炎;1例(儿童)为过敏性紫癜。他们表现出以下外周血管表现:间歇性跛行、雷诺现象、深静脉血栓形成和血栓性静脉炎各占10%;指端溃疡和营养改变占20%;手足发绀和干性坏疽占30%。60%的患者有肾脏受累。初次就诊时平均BVAS为11.5±6.57。在疾病过程中,所有患者的疾病活动度在最后一次就诊时显著改善(2.6±2.22)(p=0.002)。关于血管外科手术在其治疗中的作用,除药物治疗外,40%的患者需要额外的手术干预。2例患者进行了小趾截肢;1例进行了胸腔镜下颈交感神经切除术;另1例需要胫骨血管成形术。

结论

伴有外周缺血表现的原发性血管炎患者需要手术治疗。其治疗主要是药物治疗,并根据诊断和症状进行个体化治疗。血管内治疗可能为高手术风险患者提供一种安全且侵入性较小的方法。交感神经切除术对那些有严重疼痛和营养改变的患者具有很高的治疗潜力。

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