This handout is for scleroderma renal crisis (src) — renin-driven htn + aki + maha in systemic sclerosis. Your care team identified this based on: systemic sclerosis (especially diffuse cutaneous, <4 yr disease) + new sbp ≥150 or ≥30 mmhg increase from baseline + aki + maha features (steen ann intern med 1990 pmid 2403473).
Other reasons your team may use this plan: acute cr rise + schistocytes on smear + low haptoglobin + elevated ldh in scleroderma patient — src pattern; scleroderma + recent prednisone >15 mg/d (or pulse steroid) → src risk (helfrich arthritis rheum 1989 pmid 2916497; steen jcr 1998); scleroderma + new htn + oliguria + headache + visual changes — src presentation.
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| captopril | 12.5 mg PO q4-6h, increase by 12.5-25 mg per dose q4-6h to BP <140/90 | PO | q4-6h titrated | Steen Ann Intern Med 1990 PMID 2403473 — landmark; short-acting allows rapid titration; continue even if Cr rises (PARADOXICAL); survival benefit from <10% to >70% at 1 yr |
| lisinopril | 20-40 mg PO daily | PO | daily | Long-acting maintenance after captopril stabilization; continue lifelong (Steen JCR 2003 PMID 12867255) |
| losartan | 50 mg PO daily titrate to 100 mg | PO | daily | EULAR 2017/2024 — ARB acceptable alternative; less efficacy data than ACEi |
| amlodipine | 5-10 mg PO daily | PO | daily | CCB acceptable adjunct; ESC/ESH 2024 |
| nicardipine | 5 mg/h IV titrate by 2.5 mg/h q5-15 min, max 15 mg/h | IV | continuous | Acute IV adjunct; use sparingly to bridge to PO ACEi |
| AVOID glucocorticoid >15 mg prednisone | AVOID | N/A | N/A | Helfrich Arthritis Rheum 1989 PMID 2916497; Steen JCR 1998 — high-dose steroid (>15 mg prednisone) precipitates SRC; AVOID in scleroderma; if needed for myositis/arthritis use lowest effective dose + monitor BP/Cr daily |
| AVOID NSAIDs and nephrotoxic agents | AVOID | N/A | N/A | NSAIDs reduce renal blood flow + worsen AKI in SRC; minimize contrast; avoid aminoglycosides |
| AVOID nitroprusside long-term | AVOID >24-48h or eGFR <30 | N/A | N/A | Cyanide accumulation + thiocyanate; SRC patients have AKI → high cyanide risk; ACEi is the definitive therapy |
| Hemodialysis if oliguric + K elevated + volume overload + uremia | Per nephrology | CRRT or HD | as needed | Steen JCR 2003 PMID 12867255 — half of dialysis-requiring SRC patients recover renal function within 6-24 mo with continued ACEi; do NOT stop ACEi on dialysis |
Plan: Scleroderma renal crisis — ACE-inhibitor FIRST and CONTINUOUS even as Cr rises (PARADOXICAL but lifesaving per Steen 1990); CCB second-line; AVOID glucocorticoid; dialysis bridge with ACEi continuation
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
Lifelong ACEi continuation even on dialysis (renal recovery up to 2 yr); rheumatology follow-up for SSc disease management; dialysis transition if persistent ESRD; renal transplant possible after 2-yr stable course; AVOID future high-dose steroid; cardiac surveillance for chronic HF; pulmonary HTN screen yearly
Guideline: Steen et al Ann Intern Med 1990 PMID 2403473 (landmark ACEi in SRC) + EULAR 2024 systemic sclerosis treatment update + EULAR 2017 (Kowal-Bielecka PMID 27941129) + 2025 ACC/AHA HTN (Whelton)