Div. Rheumatology, Department of Experimental and Clinical Medicine, AOU Careggi, University of Florence, Florence, Italy.
Div. Rheumatology, Department of Experimental and Clinical Medicine, AOU Careggi, University of Florence, Florence, Italy.
Eur J Intern Med. 2021 May;87:51-58. doi: 10.1016/j.ejim.2021.01.022. Epub 2021 Feb 5.
In Systemic Sclerosis (SSc), vasculopathy is the background of major vascular complications (MVCs), like digital ulcers (DUs), pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC). We aimed to identify the predictors and to test the primary preventive effect of vasoactive/vasodilating drugs (VVD) for the development of MVCs in SSc MVCs-naïve patients.
patients fulfilling the ACR/EULAR 2013 classification criteria for SSc without history of MVCs were eligible. Data about clinical manifestations, laboratory and instrumental assessments and treatments were retrospectively collected at baseline and latest available follow-up.
134 SSc patients were enrolled (mean age 56.5 years ± 14.2, females 88.1%, limited subset 61.9%, ACA positivity 60.4%). In a mean of 43 ± 19 months of follow-up 12 (9.0%) patients developed at least 1 MVC (10 DU, 2 PAH and 1 SRC). Dyspnoea and arthritis at baseline were independent predictors for MVCs development (p = 0.012, and p = 0.002 respectively). No primary preventive effect of VVD on MVCs development was found. However, sildenafil reduced the renal resistive index increase (p = 0.042) and alprostadil slowed the DLco decline (p = 0.029). Both iloprost and angiotensin-receptor blockers (ARBs) delayed MVCs development, while angiotensin converting enzyme inhibitors (ACEi) determined an earlier onset of such MCVs.
in SSc patients, our data confirm the role of arthritis and dyspnea as independent predictors of major vascular complications, in particular in MVCs-naïve patients. Prostanoids, sildenafil and ARBs, even in absence of a primary preventive action, might help in slowing disease progression and postponing the onset of MVCs.
在系统性硬化症（SSc）中，血管病变是主要血管并发症（MVCs）的背景，如指溃疡（DU）、肺动脉高压（PAH）和硬皮病肾危象（SRC）。我们旨在确定预测因子，并测试血管活性/血管扩张药物（VVD）对 SSc MVCs 初治患者 MVCs 发展的主要预防作用。
符合 ACR/EULAR 2013 年 SSc 分类标准且无 MVCs 病史的患者符合条件。回顾性收集基线和最新随访时的临床表现、实验室和仪器评估以及治疗数据。
共纳入 134 例 SSc 患者（平均年龄 56.5 岁±14.2，女性 88.1%，局限性亚组 61.9%，ACA 阳性 60.4%）。在平均 43±19 个月的随访中，12 例（9.0%）患者至少发生了 1 次 MVC（10 例 DU、2 例 PAH 和 1 例 SRC）。基线时呼吸困难和关节炎是 MVCs 发展的独立预测因子（p=0.012 和 p=0.002）。未发现 VVD 对 MVCs 发展有主要预防作用。然而，西地那非降低了肾阻力指数的增加（p=0.042），前列地尔减缓了 DLco 的下降（p=0.029）。伊洛前列素和血管紧张素受体阻滞剂（ARB）均延迟了 MVCs 的发生，而血管紧张素转换酶抑制剂（ACEi）则导致 MVCs 更早发生。
在 SSc 患者中，我们的数据证实关节炎和呼吸困难是 MVCs 的独立预测因子，尤其是在 MVCs 初治患者中。前列腺素、西地那非和 ARB 即使没有主要的预防作用，也可能有助于减缓疾病进展并推迟 MVCs 的发生。
