Division of Rheumatology, Immunology and Allergy, MedStar Georgetown University Hospital, Washington, DC 20007, USA.
Curr Opin Rheumatol. 2012 Nov;24(6):669-76. doi: 10.1097/BOR.0b013e3283588dcf.
Renal disease remains an important cause of morbidity and mortality in scleroderma. The spectrum of renal complications in systemic sclerosis includes scleroderma renal crisis (SRC), normotensive renal crisis, antineutrophil cytoplasmic antibodies-associated glomerulonephritis, penacillamine-associated renal disease, and reduced renal functional reserves manifested by proteinuria, microalbuminuria, or isolated reduction in glomerular filtration rate. The purpose of this review is to provide a concise and up-to-date review of the evaluation, risk stratification, pathogenesis, and management of scleroderma-associated renal disease.
Although SRC survival has significantly improved, mortality of this complication remains high outside of specialized centers. Recent data demonstrate strong associations between anti-RNA polymerase III antibodies and SRC. Subclinical renal impairment affects approximately 50% of scleroderma patients and may be associated with other vascular manifestations. Subclinical renal involvement rarely progresses to end-stage renal failure; however, recent studies suggest it may predict mortality in patients with other vasculopathic manifestations.
Testing for anti-RNA polymerase III antibodies should be incorporated into clinical care to identify patients at high risk for SRC. Recommendations from European League Against Rheumatism (EULAR), EULAR Scleroderma Trials and Research, and the Scleroderma Clinical Trials Consortium confirm angiotensin-converting enzyme inhibitors as first-line therapy for SRC, and give recommendations for second-line agents.
肾脏疾病仍然是硬皮病患者发病率和死亡率的重要原因。系统性硬化症的肾脏并发症包括硬皮病肾危象（SRC）、血压正常性肾危象、抗中性粒细胞胞浆抗体相关性肾小球肾炎、青霉胺相关性肾疾病和蛋白尿、微量白蛋白尿或肾小球滤过率单独降低导致的肾功能储备降低。本文的目的是提供对硬皮病相关肾脏疾病的评估、风险分层、发病机制和管理的简明和最新综述。
尽管 SRC 的存活率显著提高，但在专门中心之外，这种并发症的死亡率仍然很高。最近的数据表明，抗 RNA 聚合酶 III 抗体与 SRC 之间存在很强的关联。大约 50%的硬皮病患者存在亚临床肾脏损害，可能与其他血管表现有关。亚临床肾脏受累很少进展为终末期肾衰竭；然而，最近的研究表明，它可能预示着有其他血管病变表现的患者的死亡率。
应将抗 RNA 聚合酶 III 抗体检测纳入临床护理，以识别 SRC 风险高的患者。欧洲抗风湿病联盟（EULAR）、EULAR 硬皮病试验和研究以及硬皮病临床试验联合会的建议证实血管紧张素转换酶抑制剂是 SRC 的一线治疗药物，并给出了二线药物的建议。
