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cardio.stemi.lupus-coronary-vasculitis.v1

STEMI — SLE-associated coronary vasculitis (immune-complex inflammation + premature CAD)

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Phase E rare-etiology variant of cardio.stemi.core.v1 — STEMI from SLE-associated coronary vasculitis (immune-complex inflammation) + premature CAD. Population: young women (20s-40s) with SLE; SLE cardiovascular RR 5-50× age-matched controls per Manzi 1997 (PMID 9329512) and Roman 2003 (PMID 14668455). Three differential drivers triaged by IVUS/OCT + serology: (1) IMMUNE-COMPLEX VASCULITIS (this engine — vessel-wall inflammation, requires pulse steroids + cyclophosphamide/MMF per EULAR 2023 PMID 36750244); (2) PREMATURE ATHEROSCLEROTIC CAD (route to parent); (3) APS THROMBOSIS (route to cardio.stemi.antiphospholipid-syndrome-related.v1 if predominant — warfarin INR 2.5-3.5 lifelong per TRAPS PMID 30196097). INHERITS parent reperfusion + DAPT + statin pathway BUT ADDS: pulse immunosuppression for active vasculitis (methylprednisolone 1 g IV × 3-5 d → prednisone taper + cyclophosphamide 500-1000 mg/m² IV monthly × 6 OR MMF 1-3 g/d alternative per ALMS PMID 19369404), lifelong hydroxychloroquine (cardiovascular benefit per Ruiz-Irastorza 2010 PMID 20132533), and APS-overlap-driven AC modification (warfarin INR 2.5-3.5 lifelong if predominant). Critical care-setting issues: rheumatology consult within 6 hours; pneumocystis prophylaxis during high-dose immunosuppression; vaccination review (live vaccines contraindicated); pregnancy + family planning counseling (HCQ safe; MMF/cyclophosphamide TERATOGENIC — switch to azathioprine 3 mo before conception; warfarin teratogenic); HCQ retinal screening annually. Sister engines: cardio.stemi.kawasaki-related.v1 (childhood vasculitis sequela vs adult active vasculitis), cardio.stemi.antiphospholipid-syndrome-related.v1 (APS thrombosis vs immune-complex inflammation; 30-50% overlap), cardio.acute-hf.lupus-myocarditis.v1 (SLE myocardium vs SLE coronaries — may coexist). Manifest pointer reuses cardio.stemi.core.v1 manifest. Design-brief pointer reuses parent. Status INTEGRATED. Authored 2026-05-15 by shard-06-cardio-acute.

Entry points (5)

  • history
    STEMI in patient with established SLE — premature CAD vs vasculitis vs APS-thrombosis triage
    stemi_in_known_sle_patient
  • symptom
    STEMI in young woman (<50) with autoimmune features (malar rash, arthritis, serositis, cytopenias) — de novo SLE consideration
    stemi_in_young_woman_with_autoimmune_features
  • imaging
    Coronary cath in young SLE patient showing diffuse disease, multivessel involvement, or vasculitic pattern (long stenoses, no discrete plaque) on IVUS/OCT
    cath_with_diffuse_disease_disproportionate_to_age_or_risk
  • lab_abnormality
    Positive ANA + anti-dsDNA + low C3/C4 + active SLE flare features in STEMI patient — immune-complex coronary inflammation suspect
    positive_ana_dsdna_low_complement_with_stemi
  • history
    Recurrent ACS / restenosis in SLE patient despite optimal DAPT + statin — vasculitic / inflammatory driver suspected
    recurrent_acs_in_sle_patient_despite_dapt_statin

Required inputs (11)

  • agerequired
    demographic • used at CONTEXT
    Young SLE patients (20s-40s, 90% women) with STEMI raise vasculitis vs premature atherosclerosis vs APS-thrombosis suspicion
  • sle_diagnosis_or_featuresrequired
    history • used at FRAME
    Established SLE (ACR/EULAR 2019 criteria) or active features (malar rash, arthritis, serositis, cytopenias, nephritis, neuropsych) — central diagnostic anchor
  • sbprequired
    vital • used at RED_FLAGS
    Hypotension + STEMI in lupus → cardiogenic shock high probability; lupus nephritis-related volume status complicates hemodynamics
  • ecgrequired
    imaging • used at INITIAL_WORKUP
    STEMI territory localization; pericarditis pattern overlap common in SLE
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Quantifies infarct burden; persistent elevation may suggest ongoing vasculitis vs single-event plaque rupture
  • creatininerequired
    lab • used at CONTEXT
    Lupus nephritis baseline + contrast nephropathy + DOAC/AC dosing
  • ana_dsdna_smith_complementrequired
    lab • used at BRANCHING_WORKUP
    SLE serology bundle: ANA (≥1:80 + clinical = SLE), anti-dsDNA + anti-Smith (specific), C3/C4 (low in active disease) — confirms active immunologic activity at index event
  • lupus_anticoagulant_acl_b2gp1required
    lab • used at BRANCHING_WORKUP
    APS antibody panel — 30-50% co-occurrence with SLE; if predominant → route to cardio.stemi.antiphospholipid-syndrome-related.v1 (warfarin INR 2.5-3.5 lifelong vs immunosuppression)
  • cor_angiorequired
    imaging • used at TREATMENT
    Diagnostic + therapeutic gold standard; lupus vasculitis often shows long diffuse stenoses, multivessel involvement, or ostial disease vs discrete plaque rupture
  • ivus_or_oct_intracoronary_imaging
    imaging • used at BRANCHING_WORKUP
    IVUS/OCT distinguishes vasculitic vessel-wall inflammation (intramural hemorrhage, eccentric thickening, no calcified plaque) from atherosclerotic plaque rupture/erosion (lipid core, thin-cap fibroatheroma) — drives vasculitis-specific immunosuppression decision
  • echo_post_admissionrequired
    imaging • used at MONITORING
    LVEF + regional wall motion + pericardial effusion (lupus pericarditis common comorbid) + valve assessment (Libman-Sacks endocarditis)

12-phase flow (10)

  1. 1FRAME
    STEMI in SLE patient with three differential drivers: (1) immune-complex coronary VASCULITIS (this engine — IVUS/OCT shows vessel-wall inflammation, active SLE serology, requires pulse immunosuppression); (2) premature ATHEROSCLEROTIC CAD (route to parent cardio.stemi.core.v1 + secondary prevention); (3) APS THROMBOSIS (route to cardio.stemi.antiphospholipid-syndrome-related.v1 if APS antibodies + thrombus disproportionate to plaque)
    inputs: sle_diagnosis_or_features
    advance: SLE-associated etiology framed
  2. 2ENTRY
    Activate cath lab; hold standard ACS bundle pending vasculitis vs atherosclerosis vs APS triage; rheumatology consult activated within 6 hours
    inputs: age
    advance: cath lab activated + rheumatology consult initiated
  3. 3CONTEXT
    SLE history (date of dx, current flare status, prior organ involvement, current immunosuppression), prior cardiovascular events, APS antibody history, current AC/antiplatelet, HCQ adherence (HCQ has cardiovascular benefit per Ruiz-Irastorza 2010 PMID 20132533)
    inputs: creatinine
    advance: SLE + cardiovascular context catalogued
  4. 4RED_FLAGS
    Cardiogenic shock (SCAI C+); concurrent active lupus organ failure (nephritis, neuropsych, hemophagocytic lymphohistiocytosis); pericardial tamponade (lupus pericarditis); CAPS (catastrophic APS overlap — multi-organ thrombosis in <1 wk, mortality 50%); Libman-Sacks endocarditis with embolic stroke
    inputs: sbp
    actions: cardiogenic_shock
    advance: red flags + concurrent lupus crisis screened
  5. 5INITIAL_WORKUP
    ECG + troponin + BMP + CBC + CXR + bedside echo (LV function + regional WMA + pericardial effusion + Libman-Sacks); SLE serology (ANA, dsDNA, Smith, C3/C4, ESR, CRP — note CRP often disproportionately low for clinical activity in pure SLE flare); APS panel (LA, aCL, β2-GPI); UA + UPCR for nephritis screen
    inputs: ecg, troponin, creatinine, echo_post_admission
    actions: acs_pathway, panel.cardiac, panel.renal, panel.coag
    advance: SLE workup initiated + cath lab booked
  6. 6BRANCHING_WORKUP
    Emergent cath with IVUS/OCT to characterize vasculitic vs atherosclerotic vs thrombotic lesion morphology; SLE serology results; APS antibody confirmation; concurrent organ involvement workup (renal biopsy if proteinuria, MRI if neuropsych); rheumatology consult formal note
    inputs: cor_angio, ivus_or_oct_intracoronary_imaging, ana_dsdna_smith_complement, lupus_anticoagulant_acl_b2gp1
    advance: lesion morphology + SLE activity + APS status documented
  7. 7TREATMENT
    Acute reperfusion: PCI guided by IVUS/OCT findings (vasculitic lesions may favor balloon angioplasty + careful stenting; CABG considered for diffuse multivessel vasculitis where PCI infeasible). PULSE IMMUNOSUPPRESSION: methylprednisolone 1 g IV × 3 d → prednisone 1 mg/kg PO daily taper; cyclophosphamide IV pulse 500-1000 mg/m² (severe organ-threatening) OR mycophenolate mofetil 1-3 g/d (ALMS PMID 19369404 alternative) OR rituximab (refractory). Continue/initiate hydroxychloroquine (cardiovascular benefit per Ruiz-Irastorza 2010 PMID 20132533). Standard ACS bundle: ASA + P2Y12 + UFH + statin
    inputs: sbp, creatinine
    actions: protocol.stemi
    advance: reperfusion + immunosuppression initiated + multidisciplinary plan documented
  8. 8DISPOSITION
    CICU 48-72 h post-reperfusion; rheumatology + cardiology + nephrology (if lupus nephritis) multidisciplinary; consider transplant referral if severe LV dysfunction
    advance: unit + multidisciplinary consult plan documented
  9. 9MONITORING
    Telemetry; serial troponin (persistent elevation may indicate ongoing vasculitis); daily SLE activity assessment (SLEDAI score, complement trend); steroid taper monitoring (hyperglycemia, infection); echo at 5-7 d for thrombus + LV reassessment; MRI for myocarditis overlap if available
    inputs: echo_post_admission
    actions: panel.cardiac
    advance: inflammation trend + LV function documented
  10. 10FOLLOWUP
    Lifelong hydroxychloroquine; immunosuppression taper per rheumatology; aggressive secondary prevention (high-intensity statin, BP control to <130/80, no smoking); cardiology + rheumatology q3-6 mo; surveillance CTA at 1 yr; pregnancy + family planning counseling (HCQ safe in pregnancy, MMF/cyclophosphamide teratogenic — switch before conception)
    advance: long-term multidisciplinary plan booked