Faculty of Medicine, McGill University, Montreal, Quebec, Canada; Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, Quebec, Canada.
Centre for Rheumatology, Royal Free London and UCL Division of Medicine, London, UK.
Autoimmun Rev. 2017 Apr;16(4):407-415. doi: 10.1016/j.autrev.2017.02.012. Epub 2017 Feb 14.
The absence of a gold standard for scleroderma renal crisis (SRC) has hindered our understanding of this problem. The objective of this scoping review was to identify the criteria used to define SRC in order to guide the development of a consensus definition for SRC.
We conducted a search in three databases: Medline, Embase and non-Ovid Pubmed. Papers were eligible for inclusion if they were full-length articles in English whose main topic was SRC or scleroderma renal disease. Two reviewers independently screened eligible papers for final study selection. Data was extracted using a customized form. A web-based survey of members of the Scleroderma Clinical Trials Consortium was used to identify unpublished definitions of SRC.
We identified 415 papers that met inclusion criteria. Forty original definitions of SRC were identified from 36 studies, 9 reviews and 2 editorials. There was significant heterogeneity in definitions. As a rule, though, in addition to new-onset hypertension and acute kidney injury, other common items used to define SRC included hypertensive encephalopathy and seizures, microangiopathic hemolytic anemia and characteristic changes on kidney biopsy. The web-based survey identified unpublished definitions of SRC that were largely consistent with the results of the published literature.
SRC was defined in a minority of studies and criteria were heterogeneous. A consensus definition of SRC is urgently needed to standardize data collection on SRC and further our understanding of this serious problem.
由于缺乏硬皮病性肾危象（SRC）的金标准，我们对这一问题的认识受到了阻碍。本研究旨在确定用于定义 SRC 的标准，以指导 SRC 的共识定义的制定。
我们在 3 个数据库（Medline、Embase 和非 Ovid Pubmed）中进行了检索。如果是关于 SRC 或硬皮病肾病的主要主题的英文全文文章，则符合纳入标准。两名评审员独立筛选合格论文以进行最终研究选择。使用定制表格提取数据。通过硬皮病临床试验联盟成员的网络调查，确定 SRC 的未发表定义。
我们确定了符合纳入标准的 415 篇论文。从 36 项研究、9 项综述和 2 篇社论中确定了 40 个 SRC 的原始定义。定义存在很大的异质性。通常情况下，除了新发高血压和急性肾损伤外，其他用于定义 SRC 的常见项目还包括高血压脑病和癫痫、微血管性溶血性贫血和肾活检的特征性改变。网络调查确定了 SRC 的未发表定义，这些定义与已发表文献的结果基本一致。
只有少数研究对 SRC 进行了定义，而且标准存在很大的异质性。迫切需要 SRC 的共识定义，以标准化 SRC 的数据收集，并进一步了解这一严重问题。
