Which statement accurately describes the presentation of scleroderma renal crisis?

• A. Abrupt onset of hypertension with rapidly progressive renal failure.
• B. Gradual blood pressure rise with stable renal function.
• C. Renal artery stenosis is the primary feature.
• D. Kidney involvement is not a feature of scleroderma.

Answer: (A)

Scleroderma renal crisis typically presents as a sudden surge in blood pressure with rapidly progressive kidney failure. This acute picture comes from a small-vessel vasculopathy in the kidneys that activates the renin–angiotensin system, leading to malignant hypertension and swift loss of renal function. You may also see signs of vascular injury such as microangiopathic hemolytic anemia in some patients, but the key clue is the abrupt, high blood pressure paired with rapid decline in kidney function, often within the first years of diffuse cutaneous systemic sclerosis. This presentation distinguishes it from a gradual rise in blood pressure with stable renal function, from mechanisms like purely renal artery stenosis, and from any notion that the kidneys aren’t involved.