Scleroderma renal crisis

被引：20
作者：
Hudson, Marie [1 ]
Ghossein, Cybele [2 ]
Steen, Virginia [3 ]
机构：
[1] McGill Univ, Lady Davis Inst, Jewish Gen Hosp, Montreal, PQ, Canada
[2] Northwestern Univ, Feinberg Sch Med, Chicago, IL 60611 USA
[3] Georgetown Univ, Med Ctr, Washington, DC 20007 USA
来源：
PRESSE MEDICALE | 2021年 / 50卷 / 01期
关键词：
SYSTEMIC-SCLEROSIS; PREGNANCY; ABNORMALITIES; PREDICTORS; RECURRENCE; MORTALITY; WOMEN;
D O I：
10.1016/j.lpm.2021.104063
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc) characterized by malignant hypertension and acute kidney injury. Historically, SRC was the leading cause of death in SSc. However, with the advent of angiotensin converting enzyme (ACE) inhibitors, mortality rates have decreased significantly. Nevertheless, one-year outcomes remain poor, with over 30% mortality and 25% of patients remaining dialysis-dependent. There is an urgent need to improve early recognition and treatment, and to identify novel treatments to improve outcomes of SRC. In this chapter, the clinical features, classification, pathophysiology, differential diagnosis, management and outcomes of SRC are presented. Specific issues relating to pregnancy, prophylactic ACE inhibition and management of essential hypertension are also discussed. (C) 2021 Elsevier Masson SAS. All rights reserved.
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页数：7
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