Scleroderma renal crisis (SRC) occurs in ∼10% of SSc patients, and is characterized by abrupt onset of hypertension, thrombotic microangiopathy (TMA), and acute kidney injury.[1] Although prognosis has improved with the use of angiotensin-converting enzyme inhibitors, 40% of patients still require dialysis, and 25% die within 1 year.[2] The pathogenesis of SSc remains poorly understood but a growing body of evidence suggests that activation of the complement system may be involved in the disease. Here, ACE is linked to systemic sclerosis.