ACE and Hypertension: In the past, the major organ complication was scleroderma renal crisis (SRC), associated with accelerated arterial hypertension and rapidly progressive deterioration of kidney function.9 Nowadays, most likely due to of the treatment with angiotensin converting enzyme (ACE) inhibitors, it is no longer the leading cause of death.7,10 Nevertheless, up to two thirds of cases require long-term dialysis and mortality remains high.3 To improve poor prognosis, early detection and immediate therapy are important.11