Division of Clinical Rheumatology, ASST Pini-CTO, Milano, Italy; Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milano, Italy.
Division of Clinical Rheumatology, ASST Pini-CTO, Milano, Italy.
Semin Arthritis Rheum. 2019 Feb;48(4):686-693. doi: 10.1016/j.semarthrit.2018.03.019. Epub 2018 Apr 4.
Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by vascular impairment, immune dysfunction and collagen deposition. Raynaud's phenomenon (RP) and digital ulcers (DU) are prominent features of SSc. Intravenous (IV) iloprost (ILO), according to the recently updated EULAR recommendations, is indicated for RP after failure of oral therapy. Moreover, IV ILO could be useful in DU healing. IV ILO is currently available mainly on the European market approved for RP secondary to SSc with 3-5 days infusion cycle. Unfortunately, data published varies regarding regimen (dosage, duration and frequency). Up to now, ILO has been studied in small cohorts of patients and in few randomized controlled trials.
A systematic review of studies on IV ILO in patients with SSc complicated by DU and RP was performed. Insufficient data were available to perform a meta-analysis according to the GRADE system. We performed a three-stage internet-based Delphi consensus exercise.
Three major indications were identified for IV ILO usage in SSc: RP non-responsive to oral therapy, DU healing, and DU prevention. IV ILO should be administered between 0.5 and 2.0ng/kg/min according to patient tolerability with a frequency depending on the indication.
Although these suggestions are supported by this expert group to be used in clinical setting, it will be necessary to formally validate the present suggestions in future clinical trials.
系统性硬化症（SSc）是一种自身免疫性慢性疾病，其特征为血管损伤、免疫功能障碍和胶原沉积。雷诺现象（RP）和指溃疡（DU）是 SSc 的突出特征。根据最近更新的 EULAR 建议，伊洛前列素（ILO）静脉注射（IV）用于口服治疗失败后的 RP。此外，IV ILO 可能对 DU 愈合有用。IV ILO 目前主要在欧洲市场上获得批准，用于 SSc 引起的 RP，输注周期为 3-5 天。不幸的是，关于方案（剂量、持续时间和频率）的数据发表情况各不相同。到目前为止，ILO 已经在少数随机对照试验和小队列患者中进行了研究。
对 SSc 并发 DU 和 RP 的 IV ILO 治疗进行了系统评价。根据 GRADE 系统，可用数据不足以进行荟萃分析。我们进行了基于互联网的三阶段 Delphi 共识研究。
确定了 IV ILO 在 SSc 中的三种主要用途：口服治疗反应不佳的 RP、DU 愈合和 DU 预防。IV ILO 应根据患者的耐受性以 0.5 至 2.0ng/kg/min 的剂量给药，频率取决于适应证。
尽管这些建议得到了专家组的支持，可在临床实践中使用，但仍有必要在未来的临床试验中正式验证这些建议。
