TY - JOUR
T1 - Scleroderma renal crisis
AU - Hudson, Marie
AU - Ghossein, Cybele
AU - Steen, Virginia
N1 - Publisher Copyright:
© 2021 Elsevier Masson SAS
PY - 2021/4
Y1 - 2021/4
N2 - 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.
AB - 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.
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U2 - 10.1016/j.lpm.2021.104063
DO - 10.1016/j.lpm.2021.104063
M3 - Short survey
C2 - 33548376
AN - SCOPUS:85102151042
SN - 0755-4982
VL - 50
JO - Presse Medicale
JF - Presse Medicale
IS - 1
M1 - 104063
ER -