Scleroderma Renal Crisis with Special Considerations for Autologous Hematopoietic Stem Cell Transplant

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, 1-year outcomes remain poor, with more than 30% mortality and 25% of patients remaining dialysis-dependent. There is an urgent need to identify novel treatments to improve outcomes of SRC. In this chapter, the clinical features, classification, pathophysiology, management, and outcomes of SRC are presented. Specific issues relating to SRC in the setting of autologous hematopoietic stem cell transplant (HSCT), including indications, eligibility criteria, risks, and mitigation strategies, are also discussed.