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.
- Start short-acting captopril promptly on clinical suspicion; do not wait for antibody results, biopsy or plasma-renin measurement.
- Titrate captopril frequently in monitored care toward the pre-crisis pressure while avoiding symptomatic hypotension; rising creatinine alone is not a reason to withdraw the ACE inhibitor.
- Add other antihypertensives when pressure remains high after ACE-inhibitor optimisation, but do not replace the ACE inhibitor with another class as the core treatment.
- Dialyse for standard urgent indications and continue ACE inhibition if tolerated; kidney recovery may occur after months, so transplant decisions are usually deferred.
- Test for TTP and other TMA, ANCA vasculitis, infection and obstruction when the phenotype is atypical, but pursue these in parallel rather than delaying captopril.
- Do not prescribe prophylactic ACE inhibitor solely to prevent renal crisis; it has not shown preventive benefit and may mask the pressure signal or associate with worse outcomes.
- After recovery, continue specialist ACE inhibition, blood-pressure self-monitoring and renal follow-up, and avoid unnecessary glucocorticoid exposure.
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, visual disturbance, confusion, seizure, retinal change, pulmonary oedema, chest pain or oliguria indicates hypertensive organ injury requiring immediate monitored treatment.
Falling platelets, schistocytes, raised LDH and low haptoglobin indicate microangiopathic haemolysis and require parallel TMA assessment.
Normotensive AKI can still be renal crisis, especially after ACE-inhibitor exposure, but carries a wider differential and often worse prognosis.
Pregnancy with hypertension and AKI requires simultaneous assessment for pre-eclampsia, renal crisis, TMA and lupus nephritis.
Headache, blurred vision, retinopathy, encephalopathy, chest pain and pulmonary oedema indicate severe vascular pressure injury.
AKI and MAHA without a large measured pressure rise remains possible, especially when ACE inhibition or cardiac dysfunction modifies presentation.
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.
- Add another antihypertensive only after ACE-inhibitor optimisation when needed, treat pulmonary oedema or seizure and involve renal and rheumatology teams at once.