Management of scleroderma renal crisis

Abstract

Purpose of review: Scleroderma renal crisis (SRC) is a life-threatening manifestation in systemic sclerosis (SSc) and is usually presented by an acute onset of severe hypertension together with an acute kidney injury. We can conceptualize SRC as a systemic syndrome with features that extend beyond the involvement of the kidney. The goal of this review is to inform clinicians about the risk factors for SRC in patients with SSc and to emphasize the importance of early identification and initiation of treatment.

Recent findings: For the past 3 decades, the use of angiotensin-converting enzyme inhibitors (ACE-I) to treat SRC, has been rightfully synonymized with a good outcome, and has changed the trajectory of mortality in SRC. Despite this, SRC still figures in one of the top four causes of mortality in patients with SSc. There is a need for additional therapeutic agents to treat SRC that is refractory to ACE-I. There has been a recent interest in combining ACE-I with endothelin receptor blockers and agents targeting the complement component 5. There is no role for using ACE-I prophylactically in high-risk patients.

Summary: Early diagnosis of SRC is the key, and early initiation of ACE-I is life-saving and associated with a better prognosis. We should consider renal transplantation in selected patients, especially those on long-term dialysis.