A 50-year-old woman with diffuse cutaneous systemic sclerosis develops a sudden severe headache and markedly elevated blood pressure, with a rapidly rising creatinine. Which complication is most important to recognise immediately?
Show answer and explanation
Answer: D Scleroderma renal crisis
Sudden severe hypertension with rapidly rising creatinine in diffuse cutaneous systemic sclerosis is scleroderma renal crisis until proven otherwise. Damaged renal arteries reduce blood flow to the kidney, which drives renin release and malignant hypertension. There may also be headache, visual disturbance, seizures, and a microangiopathic haemolytic anaemia with low platelets. It is most likely early in diffuse disease, with anti-RNA polymerase III antibodies, and after high-dose corticosteroids, so prednisolone above about 15 mg/day should be avoided where possible. Treatment is an ACE inhibitor (classically captopril, increased quickly). It is continued even if she needs dialysis, because kidney function can recover over months. Before ACE inhibitors, renal crisis was usually fatal. Monitor blood pressure and creatinine closely, and involve renal and rheumatology specialists early.
- Key clue
- New severe hypertension with acute kidney injury in diffuse systemic sclerosis
- What it means
- Scleroderma renal crisis
- What to do
- Start an ACE inhibitor urgently (even with renal impairment, given the specific evidence base in this condition), monitor blood pressure and renal function closely, and involve nephrology and rheumatology emergently
Why the other options are less appropriate
- A Raynaud's phenomenon is digital vasospasm. It does not cause severe hypertension or acute kidney injury.
- B New severe hypertension with AKI in diffuse systemic sclerosis must be treated as renal crisis, not put down to essential hypertension.
- C The headache here is caused by the hypertensive emergency. Ignoring the kidney and blood pressure findings would miss a life-threatening complication.
- E This affects the kidneys and the whole body, not just the skin.
Source: EULAR recommendations for the treatment of systemic sclerosis