Systemic sclerosis (SSc) is an autoimmune disease characterized by immune system activation, microvascular damage and fibrosis of skin and internal organs. Renal manifestations of SSc include scleroderma renal crisis (SRC), normotensive scleroderma renal crisis, MPO-ANCA-associated glomerulonephritis and vasculitis, penicillamine-associated renal disease, anti-phospholipid-associated nephropathy, isolated reduced glomerular filtration rate, reduced renal functional reserve and scleroderma-associated vasculopathy manifested by abnormal renal vascular resistance indices (RRI).