Systemic sclerosis (scleroderma) has a strong predilection in women, with onset typically between ages 30-50 years. Scleroderma kidney disease occurs in 60%-70% of patients with systemic scleroderma, mostly in those with a diffuse, rather than limited, form of cutaneous scleroderma. Kidney disease is evident by hypertension, proteinuria, and loss of glomerular filtration rate. Scleroderma kidney crisis develops in about 10%-20%, and presents as new onset malignant hypertension with acute kidney injury.