Cochin Hospital, INSERM U1016, Paris Descartes University, Paris, France.
University Hospital Zurich, Zurich, Switzerland.
Arthritis Rheumatol. 2018 Feb;70(2):162-170. doi: 10.1002/art.40377. Epub 2018 Jan 22.
Microcirculation impairment and related vasculopathy are hallmarks of systemic sclerosis (SSc). Digital ulceration is second only to Raynaud's phenomenon as a vascular complication occurring in patients with SSc. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers, and it is reasonable to question whether the outcomes of such patients might be different from those of patients who are not affected. Recently, several registries have provided relevant information about digital ulcers. Male sex and severe skin disease appear to be the main associated factors observed in several registries. However, limitations of those studies are the differences in the definitions of digital ulcers and organ involvement. Few longitudinal studies are available, and the more robust data from the European League Against Rheumatism Scleroderma Trial and Research cohort suggested worse outcomes in patients with a history of digital ulcers but could not demonstrate that a history of digital ulcers can predict additional vascular complications such as pulmonary arterial hypertension, heart failure, or renal crisis. Nevertheless, the autopsy studies published many years ago and the more recent longitudinal biomarker studies support the concept of generalized vasculopathy and a potential association between various cardiovascular complications. It is expected that with the availability of several structured registries, identification of a vascular profile or vascular phenotype will be addressed using more robust data in the near future.
微循环损伤和相关血管病变是系统性硬化症（SSc）的特征。与雷诺现象（Raynaud's phenomenon）相比，指溃疡是 SSc 患者发生的血管并发症中仅次于后者的第二大并发症。指溃疡会引起疼痛和残疾。此外，患者可能会反复发作指溃疡，因此有理由质疑这些患者的结局是否与未受影响的患者不同。最近，几个登记处提供了有关指溃疡的相关信息。几项登记处观察到，男性和严重的皮肤疾病似乎是主要的相关因素。然而，这些研究的局限性在于指溃疡和器官受累的定义存在差异。只有少数纵向研究可用，而来自欧洲抗风湿病联盟硬皮病试验和研究队列的更可靠数据表明，有指溃疡病史的患者结局更差，但无法证明指溃疡病史可以预测肺动脉高压、心力衰竭或肾危象等其他血管并发症。尽管如此，多年前发表的尸检研究和最近的纵向生物标志物研究支持广泛血管病变的概念，并支持各种心血管并发症之间存在潜在关联。预计随着几个结构化登记处的出现，使用更可靠的数据，在不久的将来将解决血管特征或血管表型的问题。
