肾脏疾病和系统性硬化症:硬皮病肾危机的最新进展。

IF 8.4 2区 医学 Q1 ALLERGY
Alice Cole, Voon H Ong, Christopher P Denton
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引用次数: 15

摘要

硬皮病肾危象(SRC)是系统性硬化症(SSc)的一种危及生命的并发症,6个月死亡率为20%。曾经是硬皮病(SSc)死亡的主要原因,它仍然是一个严重的并发症,通常需要对受影响的患者进行三级护理。随着血管紧张素转换酶抑制剂(ACEi)治疗的出现,肾脏预后有了显著改善,但由于缺乏预防措施,以及尽管有最好的医疗管理,患者仍可能迅速恶化,SRC对临床医生来说仍然是一个不稳定的挑战。跨越数十年的大型队列研究已经能够清楚地识别表型,特别是在发生SRC的风险中,从而可以加强对这些个体的监测和早期识别。新的尿液生物标志物可能为SRC患者的早期识别和治疗反应提供新的窗口。多项研究表明,补体通路在SRC中的活性增加,一些轶事病例在ACEi和治疗性血浆交换(TPE)不成功的情况下,对eculizumab治疗表现出血清学反应。内皮素-1阻断是其他SSc血管病变的一种治疗策略,已显示出作为靶点的潜力,但临床试验尚未显示出明确的治疗益处。对于SRC的管理有明确的指导方针,以规范治疗并促进风湿病医生和肾脏医生之间的早期合作。肾移植后的预后有所改善,但SRC的死亡率仍然很高,这表明需要继续探索诱发和加重SRC的机制,以开发新的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis.

Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis.

Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a mortality of 20% at 6 months. Once the leading cause of mortality in scleroderma (SSc), it remains a serious complication, often necessitating level three care for patients affected. Whilst renal outcomes have significantly improved following the advent of angiotensin-converting enzyme inhibitor (ACEi) therapy, SRC remains a precarious challenge for clinicians, due to lack of preventative measures and the fact that patients can rapidly decline despite best medical management. Large cohort studies spanning decades have allowed clear identification of phenotypes particularly at risk of developing SRC thus allowing enhanced monitoring and early identification in those individuals. Novel urinary biomarkers for renal disease in SSc may offer a new window for early identification of SRC patients and response to treatment. Multiple studies have demonstrated increased activity of complement pathways in SRC with some anecdotal cases exhibiting serological response to treatment with eculizumab where ACEi and therapeutic plasma exchange (TPE) were not successful. Endothelin-1 blockade, a therapeutic strategy in other SSc vasculopathies, has shown potential as a target but clinical trials are yet to show a clear treatment benefit. Clear guidelines for the management of SRC are in place to standardise care and facilitate early collaboration between rheumatology and renal physicians. Outcomes following renal transplant have improved but the mortality of SRC remains high, indicating the need for continued exploration of the mechanisms precipitating and exacerbating SRC in order to develop novel therapies.

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来源期刊
CiteScore
22.30
自引率
1.10%
发文量
58
审稿时长
6-12 weeks
期刊介绍: Clinical Reviews in Allergy & Immunology is a scholarly journal that focuses on the advancement of clinical management in allergic and immunologic diseases. The journal publishes both scholarly reviews and experimental papers that address the current state of managing these diseases, placing new data into perspective. Each issue of the journal is dedicated to a specific theme of critical importance to allergists and immunologists, aiming to provide a comprehensive understanding of the subject matter for a wide readership. The journal is particularly helpful in explaining how novel data impacts clinical management, along with advancements such as standardized protocols for allergy skin testing and challenge procedures, as well as improved understanding of cell biology. Ultimately, the journal aims to contribute to the improvement of care and management for patients with immune-mediated diseases.
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