Scleroderma renal crisis - 10/03/21

Doi : 10.1016/j.lpm.2021.104063

Marie Hudson ^a,⁎, Cybele Ghossein ^b, Virginia Steen ^c
^a Jewish General Hospital, Lady Davis Institute, McGill University, Montreal, Canada
^b Feinberg School of Medicine, Northwestern University, Chicago, USA
^c Georgetown University Medical Center, Washington (DC), USA

^⁎Corresponding author.

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Abstract

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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