Synopsis
Recognise scleroderma renal crisis before irreversible kidney and vascular injury, start and titrate ACE inhibition immediately, manage dialysis and microangiopathy, and distinguish mimics without delaying life-saving treatment.
- Suspect renal crisis in systemic sclerosis when blood pressure rises relative to personal baseline with AKI, headache, retinopathy, encephalopathy, heart failure or MAHA.
- Risk is highest in early rapidly progressive diffuse cutaneous disease, anti-RNA-polymerase-III positivity and recent higher-dose glucocorticoid exposure.
- Measure pressure in both arms, repeat manually, compare with previous readings and obtain creatinine, potassium, urine, blood film, platelets, LDH and haptoglobin immediately.
Key red flags
A systolic pressure rise of at least 20 mmHg or diastolic rise of at least 10 mmHg from usual can be important even when the absolute value appears modest.
Headache, blurred vision, retinopathy, encephalopathy, chest pain and pulmonary oedema indicate severe vascular pressure injury.
Investigation priorities
Confirm trajectory, guide captopril titration and identify hypertensive organ risk.
Management branches
Systemic sclerosis is accompanied by a relative pressure rise and acute kidney or hypertensive organ injury.
- Admit to monitored care, repeat pressure, assess urine output, fluid, neurological, retinal and cardiac status, and send renal, potassium and haemolysis studies.
- Give short-acting captopril immediately unless a true contraindication exists and titrate every several hours toward the previous pressure while watching symptoms, potassium and perfusion.
Key medicines
CaptoprilGive 6.25–12.5 mg orally immediately, then repeat and titrate every four to eight hours under monitored specialist care; doses may be increased rapidly toward 25–50 mg three times daily as pressure and potassium permit.
Amlodipine add-onStart 5 mg orally once daily and increase to 10 mg once daily if required after ACE-inhibitor optimisation and according to pressure response.