Diagnosis
Clinical Features
- New severe hypertension (often > 150/90 in a previously normotensive patient — a small rise of even 30 mmHg is significant)
- Acute kidney injury (rising creatinine, oliguria)
- Headache, blurred vision, fits, encephalopathy
- Microangiopathic haemolytic anaemia (schistocytes, raised LDH, low haptoglobin, thrombocytopenia)
- Acute pulmonary oedema, dyspnoea, hypertensive heart failure
- Normotensive SRC exists (10%) — usually with anti-U3-RNP, often with cardiac involvement, worse prognosis
Bloods
- Creatinine ↑
- Hb ↓, schistocytes, LDH ↑
- Platelets ↓
- Renin extremely ↑ (helpful when atypical)
- Urinalysis: mild proteinuria, bland sediment (or modest haematuria)
Imaging
- Renal USS — usually normal-sized kidneys; doppler patent
- Echo for pericardial effusion, RV dysfunction (PH worsens prognosis)
DIFFERENTIAL: malignant hypertension of any cause, atypical HUS, TTP, vasculitis, scleroderma overlap with ANCA vasculitis — biopsy occasionally needed once safe.
BIOPSY (when safe — usually deferred):
- 'Onion-skin' arteriolar hyperplasia
- Fibrinoid necrosis
- Glomerular ischaemia
- Cortical infarcts
- Thrombotic microangiopathy
Management
1. Urgent ACE Inhibition
- CAPTOPRIL is preferred — short half-life, rapid titration
- Start 6.25 mg orally, double every 4–6 h until BP target reached (often 75–150 mg/day total)
- Aim systolic BP fall of 20 mmHg/day — NOT rapid normalisation (risk of cerebral hypoperfusion)
- Continue ACEI even if creatinine rises — withdrawing ACEI worsens outcome
- Switch to long-acting ACEI (ramipril, lisinopril) once stable
2. SUPPORTIVE BP CONTROL (only if ACEI alone insufficient):
- Add calcium channel blocker (amlodipine, or IV nicardipine)
- Doxazosin if persistent hypertension
- AVOID beta-blockers as monotherapy (worsen Raynaud's)
- AVOID IV labetalol or rapid IV antihypertensives unless emergency neurology
3. AKI Care
- Strict fluid balance — many patients are euvolaemic / overloaded
- Avoid nephrotoxins; review all drugs (especially NSAIDs, ciclosporin)
- Daily U&E, FBC, LDH
- Early dialysis if oliguric, fluid-overloaded, hyperkalaemic — do not delay
- PD or HD both used; access often difficult due to vasculopathy and skin disease
4. Adjunctive / Disease-modifying
- Stop or rapidly taper corticosteroids (after discussion with rheumatology) — but NEVER abruptly if Addisonian risk
- Consider rituximab, mycophenolate, or cyclophosphamide for the underlying scleroderma (rheumatology decision)
- Complement testing if features suggest aHUS — eculizumab considered in refractory MAHA + AKI despite optimal ACEI (case-series only)
5. Recovery Phase
- Continue ACEI lifelong at maximum tolerated dose
- Home BP monitoring 1–2 × daily
- Dialysis-dependent patients may recover over 6–18 months — do NOT list for transplant until 24 months of dialysis dependence have elapsed
- Pregnancy after SRC must be planned with high-risk obstetrics, renal and rheumatology
KEY PREVENTION MESSAGE: every dcSSc patient should be educated about home BP monitoring, the risk of high-dose steroids, and to seek same-day review for new headache, breathlessness or BP rise.
