Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Rheumatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
Department of Medicine at St Vincent's Hospital, University of Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia; Department of Cardiology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, VIC 3065, Australia.
Semin Arthritis Rheum. 2019 Apr;48(5):874-887. doi: 10.1016/j.semarthrit.2018.07.008. Epub 2018 Jul 31.
Clinically evident primary heart involvement due to systemic sclerosis (SHI) is considered a poor prognostic factor and is a leading cause of systemic sclerosis (SSc) related death. Yet, there remains no consensus definition of SHI and poor understanding of the natural history and risk factors for the development of SHI.
We performed a scoping literature review of published articles with a primary focus of SHI to capture previously used definitions of SHI and items used to measure SHI. Any factors reported to be associated with an increased risk of SHI were recorded.
Of the 2436 records identified in a search of MEDLINE, EMBASE and PubMed databases, 295 were included in the final scoping review. Analysis of the literature revealed studies of variable quality, generally low patient numbers and highly heterogeneous definitions of SHI within studies. There is no clear consensus from the literature as to the scope of SHI and the prognostic significance of sub-clinical investigation abnormalities commonly detected.
The lack of a standardised definition of SHI remains a significant unmet need in SSc. The results of this review will assist in the development of consensus classification criteria to enable more accurate quantification of the burden of SHI, identification of factors associated with increased risk of developing SHI, and evaluation of the efficacy of any novel therapeutic strategies.
由于系统性硬化症（SSc）引起的临床明显原发性心脏受累（SHI）被认为是预后不良的因素，也是系统性硬化症（SSc）相关死亡的主要原因。然而，目前尚没有 SHI 的共识定义，对 SHI 的自然史和危险因素也缺乏了解。
我们对已发表的主要关注 SHI 的文献进行了范围广泛的文献回顾，以捕捉先前使用的 SHI 定义和用于测量 SHI 的项目。记录了报告与 SHI 风险增加相关的任何因素。
在对 MEDLINE、EMBASE 和 PubMed 数据库的搜索中，共确定了 2436 条记录，其中 295 条记录纳入最终的范围综述。对文献的分析显示，研究质量参差不齐，患者数量普遍较少，研究中 SHI 的定义高度异质。文献中没有明确的共识，即 SHI 的范围以及通常检测到的亚临床异常的预后意义。
缺乏 SHI 的标准化定义仍然是 SSc 中未满足的重要需求。本综述的结果将有助于制定共识分类标准，以便更准确地量化 SHI 的负担，确定与 SHI 风险增加相关的因素，并评估任何新的治疗策略的疗效。
