Division of Rheumatology, Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy.
Apheresis Unit, Department of Transfusion Medicine, University Hospital of Padova, Via Giustiniani 2, 35128 Padova, Italy.
Autoimmun Rev. 2018 Sep;17(9):882-889. doi: 10.1016/j.autrev.2018.03.012. Epub 2018 Jul 10.
Scleroderma renal crisis (SRC) is an uncommon but still life-threatening manifestation of systemic sclerosis (SSc). The incidence of SRC has decreased in the last few decades, probably due to a widespread use of vasodilators in SSc patients. It is well-recognized that exposure to different drugs can trigger SRC (corticosteroids, cyclosporine) or might prevent its occurrence (iloprost, calcium channel blockers). The prognosis of this life-threatening manifestation has not substantially improved since 1980s, when ACE-inhibitors were introduced in its treatment. ACE-inhibitors remain the mainstay in the therapy of SRC due to their efficacy in controlling malignant hypertension; indeed, the prognosis largely depends on the rapid improvement of the ongoing renal ischemia. Calcium-channel blockers and in third line diuretics and alpha-blockers should be used as additional therapy if blood pressure control remains suboptimal despite maximum tolerated doses of ACE-inhibitors. Given the growing evidence on the role of complement activation and endothelin-1 in the pathogenesis of SRC, recent case-series and case reports have suggested the use of C5-inhibitors and endothelin receptor antagonists in the therapy of SRC, mainly in the refractory cases. Plasma-exchange seems to give some benefits in patients with SRC and microangiopathy or intolerant to ACE-inhibitors. Renal transplantation is the last treatment option and its outcome is similar to that reported in other connective tissue disorders, with a 5-year patient survival rate of about 82%. In this review we summarize the current knowledge in the treatment of SRC.
硬皮病肾危象（SRC）是一种罕见但仍能威胁生命的系统性硬化症（SSc）表现。在过去的几十年中，SRC 的发病率有所下降，这可能是由于在 SSc 患者中广泛使用了血管扩张剂。人们已经认识到，接触不同的药物会引发 SRC（皮质类固醇、环孢素）或可能预防其发生（伊洛前列素、钙通道阻滞剂）。自 20 世纪 80 年代引入 ACE 抑制剂治疗以来，这种危及生命的表现的预后并没有得到实质性改善。ACE 抑制剂仍然是 SRC 治疗的主要方法，因为它们在控制恶性高血压方面有效；事实上，预后在很大程度上取决于正在进行的肾缺血的迅速改善。如果尽管使用了最大耐受剂量的 ACE 抑制剂，但血压控制仍不理想，则应使用钙通道阻滞剂和三线利尿剂和α受体阻滞剂作为额外的治疗方法。鉴于补体激活和内皮素-1在 SRC 发病机制中的作用的证据越来越多，最近的病例系列和病例报告表明，C5 抑制剂和内皮素受体拮抗剂可用于 SRC 的治疗，主要用于难治性病例。血浆置换似乎对 SRC 合并微血管病变或不能耐受 ACE 抑制剂的患者有一定益处。肾移植是最后的治疗选择，其结果与其他结缔组织疾病相似，5 年患者生存率约为 82%。在这篇综述中，我们总结了 SRC 治疗的最新知识。
