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cardio.hypertensive-emergency.scleroderma-renal-crisis.v1PRODUCTION
cardio.hypertensive-emergency.scleroderma-renal-crisis.v1

Scleroderma renal crisis (SRC) — renin-driven HTN + AKI + MAHA in systemic sclerosis

cardiologyacuteadult
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10/12 authored

Canonical 12-phase frame with authored status for this dossier.

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Detailed

Scleroderma renal crisis (SRC) = acute renin-driven malignant HTN + AKI + MAHA in patient with systemic sclerosis (especially diffuse cutaneous SSc + early disease + recent high-dose steroid). Pathophysiology: scleroderma vasculopathy → glomerular hypoperfusion → renin surge → angiotensin II → severe vasoconstriction + hyperreninemia → microangiopathy. Pharmacology pivot: ACE INHIBITOR FIRST AND CONTINUOUS even as Cr rises (PARADOXICAL — Steen Ann Intern Med 1990 PMID 2403473 changed survival from <10% to >70% at 1 yr); ARB if ACEi-intolerant; CCB for additional control; AVOID glucocorticoid (precipitates SRC). Dialysis if needed (often transient — half recover in 6-24 mo). Route to parent engine for shared HTN-emergency arc; this dossier owns the renin-driven pharmacology + MAHA workup + scleroderma-specific perspective.

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SRC criteria met (scleroderma + new HTN + AKI ± MAHA)

Patient inputs (12)

SRC peak in 40-60 y; older patients have worse survival (Penn Rheumatology 2007)

Diffuse cutaneous SSc accounts for 80% of SRC; limited cutaneous SSc rare; disease duration <4 yr highest risk (Steen Ann Intern Med 1990)

SRC criteria require ≥30 mmHg SBP increase from baseline — patients with baseline 110/70 may meet criteria at 140/90

AKI is defining feature (acute Cr rise); also drives drug dosing; PARADOXICAL — continue ACEi even if Cr rises (Steen 1990)

Schistocytes + thrombocytopenia define MAHA pattern in SRC

Low haptoglobin supports MAHA hemolysis

Elevated LDH supports hemolysis + tissue damage

Mild proteinuria + microscopic hematuria common; granular casts; differentiate from glomerulonephritis (heavy proteinuria + RBC casts more typical of GN)

Defines crisis threshold (≥150 OR ≥30 mmHg above baseline); drives ACEi titration rate

Component of MAP; classic DBP elevation ≥85 in SRC

Pulmonary hypertension common in SSc; PASP elevation drives prognosis + treatment selection

Markedly elevated renin in SRC supports renin-driven mechanism (research-grade marker; not always available; treatment not delayed)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (6)

6 need judgement
  • informationallife_threateningsrc_with_oliguric_AKI_and_hyperK
    SRC + oliguria + K >6 + uremic symptoms — urgent dialysis indication while ACEi continued
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningsrc_recurrence_after_acei_taper
    BP rebound + AKI + MAHA recurrence after ACEi dose reduction or omission — SRC recurrence (~20% of patients)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningsrc_after_high_dose_steroid_administration
    New SRC episode within days-weeks of starting high-dose prednisone (>15 mg) for SSc-related arthritis/myositis — iatrogenic SRC
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningsrc_with_PRES_or_stroke
    SRC + new neurologic symptoms (HA, vision change, AMS, focal deficit) — PRES (posterior reversible encephalopathy syndrome) or stroke from severe HTN
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningsrc_with_pulmonary_edema_or_cardiac_involvement
    SRC + pulmonary edema + new LV dysfunction — hypertensive cardiomyopathy or scleroderma cardiac involvement
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresrc_with_MAHA_severe_anemia
    SRC + Hgb <7 + active hemolysis (haptoglobin undetectable, LDH >2× ULN, schistocytes >5/hpf) — severe MAHA
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

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
axis: scleroderma_renal_crisis_acei_first_pharmacology
Selected axis "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" by default fallback (first axis)
  • captopril
    first line
    ace_inhibitor_short_acting
    12.5 mg PO q4-6h, increase by 12.5-25 mg per dose q4-6h to BP <140/90 • PO • q4-6h titrated
    triggers: scleroderma_renal_crisis
    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
    rxcui 1998
  • lisinopril
    add on
    ace_inhibitor_long_acting
    20-40 mg PO daily • PO • daily
    triggers: SRC_chronic_management_after_acute_stabilization
    Long-acting maintenance after captopril stabilization; continue lifelong (Steen JCR 2003 PMID 12867255)
    rxcui 29046
  • losartan
    contraindication substitute
    arb
    50 mg PO daily titrate to 100 mg • PO • daily
    triggers: ACEi_intolerant_cough_or_angioedema
    EULAR 2017/2024 — ARB acceptable alternative; less efficacy data than ACEi
    rxcui 52175
  • amlodipine
    add on
    DHP_CCB
    5-10 mg PO daily • PO • daily
    triggers: BP_not_at_target_on_max_ACEi
    CCB acceptable adjunct; ESC/ESH 2024
    rxcui 17767
  • nicardipine
    second line
    DHP_CCB_IV
    5 mg/h IV titrate by 2.5 mg/h q5-15 min, max 15 mg/h • IV • continuous
    triggers: SRC_with_severe_HTN_unable_to_take_PO, transition_period_from_PO_captopril
    Acute IV adjunct; use sparingly to bridge to PO ACEi
    rxcui 7396
  • AVOID glucocorticoid >15 mg prednisone
    contraindication substitute
    do_not_use
    AVOID • N/A • N/A
    triggers: scleroderma_diagnosis
    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
    contraindication substitute
    do_not_use
    AVOID • N/A • N/A
    triggers: scleroderma_or_SRC_diagnosis
    NSAIDs reduce renal blood flow + worsen AKI in SRC; minimize contrast; avoid aminoglycosides
  • AVOID nitroprusside long-term
    contraindication substitute
    do_not_use
    AVOID >24-48h or eGFR <30 • N/A • N/A
    triggers: SRC_with_severe_AKI
    Cyanide accumulation + thiocyanate; SRC patients have AKI → high cyanide risk; ACEi is the definitive therapy
  • Hemodialysis if oliguric + K elevated + volume overload + uremia
    rescue
    renal_replacement_therapy
    Per nephrology • CRRT or HD • as needed
    triggers: SRC_with_oliguric_AKI_uremia_hyperK
    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

outpatient playbook — drug actions (4)

  1. 1. lifelong ACEi
    rxcui 29046
    Lisinopril 20-40 mg PO daily • PO • daily
    trigger: SRC history
    Steen JCR 2003 — lifelong even on dialysis
  2. 2. CCB ± diuretic
    rxcui 17767
    Amlodipine 5-10 + chlorthalidone 12.5-25 • PO • daily
    trigger: BP target
    ACC/AHA 2025
  3. 3. mycophenolate or other SSc DMARD per rheumatology
    Per rheumatology • PO • per regimen
    trigger: SSc disease activity
    EULAR 2024 — non-steroid disease modification
  4. 4. PH-targeted therapy if PAH develops
    Per pulmonology • PO/inhaled/IV • per regimen
    trigger: PAH on RHC
    EULAR 2024

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: 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); 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).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Scleroderma renal crisis (SRC) — renin-driven HTN + AKI + MAHA in systemic sclerosis** (cardio.hypertensive-emergency.scleroderma-renal-crisis.v1).
Scope: Scleroderma renal crisis (SRC) = acute renin-driven malignant HTN + AKI + MAHA in patient with systemic sclerosis (especially diffuse cutaneous SSc + early disease + recent high-dose steroid). Pathophysiology: scleroderma vasculopathy → glomerular hypoperfusion → renin surge → angiotensin II → severe vasoconstriction + hyperreninemia → microangiopathy. Pharmacology pivot: ACE INHIBITOR FIRST AND CONTINUOUS even as Cr rises (PARADOXICAL — Steen Ann Intern Med 1990 PMID 2403473 changed survival from <10% to >70% at 1 yr); ARB if ACEi-intolerant; CCB for additional control; AVOID glucocorticoid (precipitates SRC). Dialysis if needed (often transient — half recover in 6-24 mo). Route to parent engine for shared HTN-emergency arc; this dossier owns the renin-driven pharmacology + MAHA workup + scleroderma-specific perspective.

No severity triggers fired against current inputs.

Plan

Regimen axis: **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**.
1. 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 (ace_inhibitor_short_acting, first line) — 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
2. lisinopril 20-40 mg PO daily PO daily (ace_inhibitor_long_acting, add on) — Long-acting maintenance after captopril stabilization; continue lifelong (Steen JCR 2003 PMID 12867255)
3. losartan 50 mg PO daily titrate to 100 mg PO daily (arb, contraindication substitute) — EULAR 2017/2024 — ARB acceptable alternative; less efficacy data than ACEi
4. amlodipine 5-10 mg PO daily PO daily (DHP_CCB, add on) — CCB acceptable adjunct; ESC/ESH 2024
5. nicardipine 5 mg/h IV titrate by 2.5 mg/h q5-15 min, max 15 mg/h IV continuous (DHP_CCB_IV, second line) — Acute IV adjunct; use sparingly to bridge to PO ACEi
6. AVOID glucocorticoid >15 mg prednisone AVOID N/A N/A (do_not_use, contraindication substitute) — 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
7. AVOID NSAIDs and nephrotoxic agents AVOID N/A N/A (do_not_use, contraindication substitute) — NSAIDs reduce renal blood flow + worsen AKI in SRC; minimize contrast; avoid aminoglycosides
8. AVOID nitroprusside long-term AVOID >24-48h or eGFR <30 N/A N/A (do_not_use, contraindication substitute) — Cyanide accumulation + thiocyanate; SRC patients have AKI → high cyanide risk; ACEi is the definitive therapy
9. Hemodialysis if oliguric + K elevated + volume overload + uremia Per nephrology CRRT or HD as needed (renal_replacement_therapy, rescue) — 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

Setting playbook (outpatient) — Lifelong rheumatology + nephrology + cardiology + PCP coordination — sustained ACEi (even on dialysis), SSc disease management, renal recovery assessment (potential dialysis weaning), pulmonary HTN screen, AVOID future steroid
10. lifelong ACEi Lisinopril 20-40 mg PO daily PO daily — SRC history (Steen JCR 2003 — lifelong even on dialysis)
11. CCB ± diuretic Amlodipine 5-10 + chlorthalidone 12.5-25 PO daily — BP target (ACC/AHA 2025)
12. mycophenolate or other SSc DMARD per rheumatology Per rheumatology PO per regimen — SSc disease activity (EULAR 2024 — non-steroid disease modification)
13. PH-targeted therapy if PAH develops Per pulmonology PO/inhaled/IV per regimen — PAH on RHC (EULAR 2024)

Non-pharmacologic actions:
- Lifelong rheumatology follow-up
- Annual echo + PFT + HRCT
- Renal transplant possible after 2-yr stable course (waitlist evaluation)
- Patient + family education
- AVOID future high-dose steroid documented in all records

AVOID / contraindication checks:
- High_dose_glucocorticoid_avoid_in_scleroderma_precipitates_SRC (Helfrich 1989 PMID 2916497; Steen JCR 1998)
- NSAID_avoid_in_SRC_worsens_AKI
- Contrast_minimize_in_SRC_AKI
- Nitroprusside_avoid_long_term_or_eGFR_below_30
- CONTINUE_ACEi_even_if_Cr_rises_in_SRC (Steen Ann Intern Med 1990 PMID 2403473) — PARADOXICAL but lifesaving

Monitoring

Regimen monitoring:
- arterial line q4-6h BP during titration (Steen 1990)
- q4-6h BMP with K and Cr attention (continue ACEi even with Cr rise)
- daily CBC with smear for MAHA trajectory
- q24-48h haptoglobin LDH indirect bili
- daily UOP and dialysis access consideration
- daily neuro exam for PRES or stroke complications
- echo at baseline and post-stabilization (cardiac involvement)
- PASP screen (PH common in SSc)

Setting (outpatient) monitoring:
- Quarterly BP + BMP + CBC
- Annual echo + PASP + PFT
- Renal recovery assessment

Follow-up plan: 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
- Close-out criterion: lifelong ACEi + rheumatology + nephrology + cardiology surveillance plan in place

Monitoring phase: A-line + q4-6h BP during titration; q4-6h BMP for K + Cr (PARADOXICAL — Cr rise often expected with ACEi in SRC; do not stop unless very severe); CBC with smear daily for MAHA trajectory; haptoglobin + LDH q24-48h; UOP + dialysis access if oliguric; serial echo for cardiac function

Disposition

Current setting: outpatient — Lifelong rheumatology + nephrology + cardiology + PCP coordination — sustained ACEi (even on dialysis), SSc disease management, renal recovery assessment (potential dialysis weaning), pulmonary HTN screen, AVOID future steroid

Disposition criteria:
- Long-term continuation; cross-link to nephrology + rheumatology + cardio.htn.core.v1 for chronic management

Escalation triggers (move to higher acuity):
- BP rebound → urgent visit
- Recurrent SRC → ED + ACEi escalation
- New PH → pulmonology
- New ILD progression → pulmonology

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] SRC + oliguria + K >6 + uremic symptoms — urgent dialysis indication while ACEi continued
- [LIFE_THREATENING] BP rebound + AKI + MAHA recurrence after ACEi dose reduction or omission — SRC recurrence (~20% of patients)
- [LIFE_THREATENING] New SRC episode within days-weeks of starting high-dose prednisone (>15 mg) for SSc-related arthritis/myositis — iatrogenic SRC

Citations

- 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) [PMID:2403473](https://pubmed.ncbi.nlm.nih.gov/2403473/)
- Cited evidence (PMID 2916497) [PMID:2916497](https://pubmed.ncbi.nlm.nih.gov/2916497/)
- Cited evidence (PMID 27941129) [PMID:27941129](https://pubmed.ncbi.nlm.nih.gov/27941129/)
- Cited evidence (PMID 17170400) [PMID:17170400](https://pubmed.ncbi.nlm.nih.gov/17170400/)
- Cited evidence (PMID 12867255) [PMID:12867255](https://pubmed.ncbi.nlm.nih.gov/12867255/)

Last reconciled with current guidelines: 2026-05-14.
References
  • 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) — PMID:2403473
  • Cited evidence (PMID 2916497) — PMID:2916497
  • Cited evidence (PMID 27941129) — PMID:27941129
  • Cited evidence (PMID 17170400) — PMID:17170400
  • Cited evidence (PMID 12867255) — PMID:12867255