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cardio.acute-hf.lupus-myocarditis.v1

ADHF in SLE myocarditis

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Phase E adjacent-disease variant of cardio.acute-hf.core.v1 — narrowed to ADHF caused by SLE myocarditis. Phenotype-first triage drives immunosuppression intensity (pulse methylprednisolone + cyclophosphamide vs mycophenolate vs rituximab/IVIG salvage). Diagnostic algorithm: SLE serology bundle (ANA + dsDNA + Smith + Ro/La + C3/C4) + APL panel + UA with protein; cardiac MRI Lake Louise 2018; EMB GOLD STANDARD if life-threatening or atypical (AHA 2020 PMID 32200645). Strongyloides serology MANDATORY before high-dose steroids if exposure history. Critical AVOID list: NSAIDs (lupus nephritis AKI); live vaccines during immunosuppression; DOACs inferior to warfarin in triple-positive APL syndrome (TRAPS PMID 30002145). Multidisciplinary co-management mandatory: rheumatology + cardiology; oncology if ICI-myocarditis overlap. Long-term GDMT 4-pillar for residual HFrEF; ICD per HRS 2017 (PMID 28219760) if EF <35 + sustained VT despite ≥3 mo GDMT + immunosuppression. Manifest pointer reuses cardio.acute-hf.core.v1 manifest. Design-brief reuses parent. Status INTEGRATED; authored 2026-05-15 by shard-06-cardio-acute (Phase E wave 22 systemic-disease).

Entry points (4)

  • symptom
    New HF symptoms in patient with established SLE or recently positive ANA + dsDNA + low complement
    hf_symptoms_with_known_sle_or_serology
  • symptom
    Young woman (15-45) with new HF + pleuritic chest pain + arthritis + rash → SLE myocarditis screen
    young_woman_with_hf_and_polyserositis
  • lab_abnormality
    ANA ≥1:80 + low C3/C4 + elevated troponin without obstructive CAD on cath
    positive_ana_with_low_complement_and_hf
  • imaging
    Cardiac MRI Lake Louise 2018 positive (T2 elevation + LGE patchy mid-wall) + positive SLE serology
    cmr_lake_louise_with_sle_serology

Required inputs (15)

  • agerequired
    demographic • used at CONTEXT
    SLE peak incidence 15-45; pediatric SLE more aggressive; late-onset SLE (>50) often milder
  • sexrequired
    demographic • used at CONTEXT
    F:M ~9:1 reproductive age; cardiac SLE may behave differently across sex
  • prior_sle_diagnosis_or_featuresrequired
    history • used at CONTEXT
    Prior ACR/EULAR-criteria SLE diagnosis or features (malar rash, arthritis, serositis, nephritis, cytopenias, neuropsychiatric)
  • cancer_with_ici_exposure
    history • used at CONTEXT
    Concurrent ICI-myocarditis must be excluded if cancer history — overlap with SLE flare changes management
  • sbprequired
    vital • used at RED_FLAGS
    Hypotension may indicate myocarditis severity, pericardial tamponade, or vasodilatory state
  • nt_probnprequired
    lab • used at INITIAL_WORKUP
    HF severity stratification + monitoring response to immunosuppression
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Reflects ongoing myocyte injury; trend daily during immunosuppression
  • creatininerequired
    lab • used at CONTEXT
    Lupus nephritis common; baseline before ACEi/ARB/NSAID + cyclophosphamide; KDIGO 2026 staging
  • ana_dsdna_smith_complementrequired
    lab • used at BRANCHING_WORKUP
    SLE confirmation (ANA ≥1:80, anti-dsDNA + anti-Smith specific) + flare activity (low C3/C4 + rising dsDNA)
  • antiphospholipid_panelrequired
    lab • used at BRANCHING_WORKUP
    Lupus anticoagulant + anti-cardiolipin + anti-β2GP1 — drives Libman-Sacks AC decision and stroke risk
  • urinalysis_with_proteinrequired
    lab • used at BRANCHING_WORKUP
    Lupus nephritis screen — proteinuria + active sediment changes management (mycophenolate vs cyclophosphamide)
  • strongyloides_serology
    lab • used at BRANCHING_WORKUP
    MANDATORY before high-dose steroids in patients with possible exposure (steroids precipitate fatal hyperinfection)
  • echo_with_strainrequired
    imaging • used at INITIAL_WORKUP
    LV dysfunction, regional wall motion, pericardial effusion, Libman-Sacks valve thickening / vegetation
  • cardiac_mri_lake_louiserequired
    imaging • used at BRANCHING_WORKUP
    Lake Louise 2018 — T2 active edema + LGE patchy mid-wall non-ischemic distribution
  • emb_if_severe
    imaging • used at BRANCHING_WORKUP
    Endomyocardial biopsy gold standard if life-threatening (CS, fulminant) or atypical presentation per AHA 2020 (PMID 32200645)

12-phase flow (12)

  1. 1FRAME
    SLE myocarditis presenting as ADHF — phenotype-first triage (immunosuppression-responsive vs concurrent ICI-myocarditis vs Libman-Sacks endocarditis with embolic complication)
    inputs: age, sex
    advance: SLE myocarditis suspected
  2. 2ENTRY
    Known SLE + new HF, or young woman with HF + serositis + arthritis, or positive ANA + low complement + troponin elevation without obstructive CAD
    inputs: age
    advance: one entry trigger present
  3. 3CONTEXT
    Demographics, prior SLE features, ICI exposure history, baseline renal/hepatic function (KDIGO 2026), pregnancy status (cyclophosphamide gonadotoxicity)
    inputs: age, sex, prior_sle_diagnosis_or_features, sbp, creatinine
    advance: context complete
  4. 4RED_FLAGS
    Cardiogenic shock (SCAI C+); fulminant myocarditis; pericardial tamponade; multi-organ SLE flare with lupus nephritis worsening on ACEi/ARB; ventricular arrhythmia
    inputs: sbp, troponin, nt_probnp
    actions: cardiogenic_shock
    advance: red flags screened or escalated
  5. 5INITIAL_WORKUP
    NT-proBNP + troponin + BMP + CBC + lactate + ECG + bedside echo with strain (LV dysfunction, regional WMA, pericardial effusion, Libman-Sacks)
    inputs: nt_probnp, troponin, creatinine, echo_with_strain
    actions: acute_pulm_edema, panel.cardiac, panel.renal
    advance: baseline workup documented
  6. 6BRANCHING_WORKUP
    SLE serology bundle (ANA + dsDNA + Smith + Ro/La + C3/C4) + APL panel + UA with protein; cardiac MRI Lake Louise; EMB if severe / atypical; STRONGYLOIDES serology mandatory before high-dose steroids
    inputs: ana_dsdna_smith_complement, antiphospholipid_panel, urinalysis_with_protein, cardiac_mri_lake_louise
    advance: SLE myocarditis confirmed or alternative diagnosis identified
  7. 7DIFFERENTIAL
    SLE myocarditis vs viral myocarditis vs ICI-myocarditis (cancer + ICI exposure overlap) vs giant-cell myocarditis vs eosinophilic myocarditis vs sarcoid
    inputs: emb_if_severe
    advance: etiology assigned + mimics excluded
  8. 8RISK_STRATIFICATION
    SLE Disease Activity Index (SLEDAI) + organ involvement count + cardiac MRI burden — drives immunosuppression intensity (cyclophosphamide vs mycophenolate vs rituximab)
    inputs: nt_probnp, troponin, creatinine
    advance: severity stratified
  9. 9TREATMENT
    Standard ADHF (gentle diuresis) + IMMUNOSUPPRESSION: pulse methylprednisolone 1 g IV daily × 3-5 d → prednisone 1 mg/kg taper; cyclophosphamide IV pulse 500-1000 mg/m² monthly × 6 for severe; mycophenolate alternative; rituximab + IVIG for refractory; AVOID NSAIDs
    inputs: sbp, creatinine, urinalysis_with_protein
    actions: protocol.cardiogenic_shock
    advance: immunosuppression + supportive plan started
  10. 10DISPOSITION
    Floor vs ICU; rheumatology + cardiology multidisciplinary mandatory; if cancer + ICI exposure → oncology join
    advance: unit + multidisciplinary team assigned
  11. 11MONITORING
    Daily troponin trend (response marker); weight + BMP + UA; weekly C3/C4 + dsDNA during steroid taper; echo at 2 wk + 3 mo; CMR at 3 mo to confirm resolution
    inputs: creatinine, nt_probnp, troponin
    actions: panel.renal
    advance: monitoring plan documented
  12. 12FOLLOWUP
    Long-term rheumatology + cardiology co-management; SLE flare prevention with HCQ + DMARD; GDMT 4-pillar for residual HFrEF; ICD eval per HRS 2017 if EF <35 + sustained VT despite ≥3 mo GDMT + immunosuppression
    advance: long-term plan booked