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cardio.stemi.behcet-coronary-vasculitis.v1

STEMI — Behçet-disease-associated coronary vasculitis (variable-vessel vasculitis with aneurysms + thrombosis)

cardiologyacuteadultacuteinpatienttransitionoutpatient
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Phase E rare-etiology variant of cardio.stemi.core.v1 — STEMI from Behçet-disease-associated coronary vasculitis (variable-vessel vasculitis with arterial + venous involvement). Population: young adults (20-40s, vasculo-Behçet predominantly young men), Mediterranean / Silk Road origin, HLA-B51 frequent (~60%); cardiac involvement 7-46%, coronary involvement 1-5% (Saadoun 2012 PMID 22588748). Behçet is the prototypical VARIABLE-VESSEL vasculitis (Chapel Hill 2012 PMID 23045170) — uniquely arteries AND veins of any size. Coronary manifestations: saccular aneurysms with intra-aneurysmal thrombosis (most common), true vasculitis (vessel-wall infiltrate), coronary spasm (less common), pericarditis / myocarditis comorbid. CRITICAL TREATMENT DIFFERENCES: (1) AVOID FIBRINOLYSIS — coronary aneurysm rupture risk; PRIMARY PCI ONLY; (2) IMMUNOSUPPRESSION IS CORE not adjunct: high-dose pulse steroids + cyclophosphamide for severe vasculo-Behçet per EULAR 2018 (Hatemi PMID 29625968) Class I; (3) COLCHICINE foundational lifelong; (4) TNF-α inhibitor (infliximab — Sfikakis 2004 PMID 15571742) for refractory or steroid-sparing; (5) AVOID aspirin monotherapy as sole antithrombotic in pure-vasculitic; (6) Cautious AC given aneurysm rupture risk — only with concurrent immunosuppression and clear thrombus. Critical care-setting issues: rheumatology consult within 6 hours; pulmonary artery aneurysm rupture is the most lethal vasculo-Behçet manifestation (massive hemoptysis); CTA chest/abdomen/pelvis within 24-48 h for extra-coronary aneurysm screen; pneumocystis prophylaxis during high-dose immunosuppression; vaccination review (live vaccines contraindicated); smoking cessation critical (major flare trigger); pregnancy + family planning counseling (colchicine compatible; cyclophosphamide / MMF teratogenic — switch to azathioprine 3 mo before conception). Sister engines: cardio.stemi.kawasaki-related.v1 (childhood vasculitis sequela vs adult active vasculitis), cardio.stemi.lupus-coronary-vasculitis.v1 (immune-complex small/medium vessel vs variable-vessel), cardio.stemi.antiphospholipid-syndrome-related.v1 (antibody-mediated thrombosis vs vasculitis with aneurysm). 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 (6)

  • history
    STEMI in patient with established Behçet disease — coronary vasculitis vs aneurysm thrombosis vs spasm triage
    stemi_in_known_behcet_patient
  • symptom
    STEMI in young man (20-40 yr; Mediterranean / Silk Road origin) with recurrent oral + genital ulcers + uveitis + skin lesions → de novo Behçet consideration
    stemi_in_young_man_with_recurrent_oral_genital_ulcers
  • imaging
    Coronary cath shows aneurysmal coronary segments (saccular, mycotic-like appearance) with intra-aneurysmal thrombus → variable-vessel vasculitis suspect; AVOID fibrinolysis given rupture risk
    cath_with_coronary_aneurysm_+_thrombus_in_behcet_suspect
  • history
    STEMI with concurrent DVT / PE / large-vessel arterial aneurysm (pulmonary, aortic) — vasculo-Behçet subtype with arterial + venous involvement
    stemi_with_concurrent_dvt_pe_or_arterial_aneurysm
  • lab_abnormality
    Positive pathergy test + HLA-B51 + active vasculitis features (elevated ESR/CRP) in young STEMI patient — Behçet diagnostic anchors
    positive_pathergy_or_hla_b51_with_acs
  • imaging
    Recurrent ACS / restenosis in Behçet patient despite optimal DAPT + statin with new or progressive aneurysm formation on serial CTA — ongoing vasculitis driver
    recurrent_acs_in_behcet_despite_dapt_with_aneurysm_progression

Required inputs (12)

  • agerequired
    demographic • used at CONTEXT
    Behçet typical onset 25-30 yr; vasculo-Behçet predominantly young men; STEMI <50 with mucocutaneous features should trigger BD workup
  • behcet_diagnosis_or_featuresrequired
    history • used at FRAME
    ISG criteria: recurrent oral ulceration ≥3/yr PLUS ≥2 of recurrent genital ulceration, eye lesions (uveitis), skin lesions (erythema nodosum, pseudofolliculitis), positive pathergy — central diagnostic anchor
  • sbprequired
    vital • used at RED_FLAGS
    Hypotension + STEMI in BD with coronary aneurysm thrombosis → cardiogenic shock high probability; rupture of coronary or extra-coronary aneurysm produces shock
  • ecgrequired
    imaging • used at INITIAL_WORKUP
    STEMI territory localization; pericarditis pattern overlap (BD pericarditis common comorbid)
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Quantifies infarct burden; persistent elevation may suggest ongoing vasculitis vs single-event thrombosis
  • creatininerequired
    lab • used at CONTEXT
    Baseline + contrast nephropathy + cyclophosphamide / MMF dose adjustment + AC dosing
  • esr_crp_ferritinrequired
    lab • used at BRANCHING_WORKUP
    Acute-phase reactants — typically elevated in active vasculo-Behçet (unlike pure SLE where CRP often spared); guides immunosuppression intensity
  • hla_b51_typing
    lab • used at BRANCHING_WORKUP
    HLA-B51 frequent (~60% Mediterranean / Silk Road); supports BD diagnosis when clinical features incomplete
  • cor_angiorequired
    imaging • used at TREATMENT
    Diagnostic + therapeutic gold standard; BD vasculitis often shows aneurysmal coronary segments (saccular), intra-aneurysmal thrombus, multivessel involvement; AVOID fibrinolysis given rupture risk
  • ivus_or_oct_intracoronary_imaging
    imaging • used at BRANCHING_WORKUP
    IVUS/OCT distinguishes vasculitic vessel-wall inflammation + aneurysm wall integrity from atherosclerotic plaque; identifies stent landing zones avoiding aneurysmal segments; drives PCI vs CABG decision
  • echo_post_admissionrequired
    imaging • used at MONITORING
    LVEF + regional wall motion + pericardial effusion (BD pericarditis common comorbid) + valve assessment + intracardiac thrombus screen
  • pathergy_uveitis_eye_exam_history
    history • used at BRANCHING_WORKUP
    Pathergy test (sterile pustule at needle prick site) + ophthalmology eye exam (anterior + posterior uveitis, retinal vasculitis) — complete BD criteria assessment

12-phase flow (10)

  1. 1FRAME
    STEMI in BD patient with three differential drivers: (1) ACTIVE CORONARY VASCULITIS with aneurysm thrombosis (this engine — IVUS/OCT shows vessel-wall inflammation + aneurysm thrombus, requires high-dose immunosuppression + careful PCI / CABG); (2) CORONARY SPASM (less common; nitrates + CCB); (3) thrombosis-predominant without active inflammation (still requires immunosuppression to prevent recurrence). AVOID fibrinolysis given aneurysm rupture risk; AVOID aspirin monotherapy as sole antithrombotic in pure-vasculitic phenotype.
    inputs: behcet_diagnosis_or_features
    advance: Behçet-associated etiology framed
  2. 2ENTRY
    Activate cath lab; AVOID fibrinolysis — primary PCI mandatory; rheumatology consult activated within 6 hours; check for concurrent vasculo-Behçet activity (DVT, PE, aortic aneurysm, neuro-Behçet)
    inputs: age
    advance: cath lab activated + rheumatology consult initiated + fibrinolysis withheld
  3. 3CONTEXT
    BD history (date of dx, current flare status, prior vascular events, current immunosuppression including colchicine adherence), prior cardiovascular events, aneurysm imaging, current AC/antiplatelet, ophthalmology + dermatology history
    inputs: creatinine
    advance: BD + cardiovascular context catalogued
  4. 4RED_FLAGS
    Cardiogenic shock (SCAI C+); CORONARY OR EXTRA-CORONARY ANEURYSM RUPTURE (catastrophic — pulmonary artery aneurysm rupture characteristic of vasculo-Behçet, abdominal aortic aneurysm); concurrent neuro-Behçet (parenchymal CNS vasculitis); pericardial tamponade; intracardiac thrombus with systemic embolization
    inputs: sbp
    actions: cardiogenic_shock
    advance: red flags + concurrent vasculo-Behçet crises screened
  5. 5INITIAL_WORKUP
    ECG + troponin + BMP + CBC + CXR (pulmonary artery aneurysm screen) + bedside echo (LV function + regional WMA + pericardial effusion + intracardiac thrombus); inflammatory markers (ESR, CRP — typically elevated in vasculo-Behçet); coagulation panel; UA; full skin + mucocutaneous + ophthalmology exam
    inputs: ecg, troponin, creatinine, esr_crp_ferritin, echo_post_admission
    actions: acs_pathway, panel.cardiac, panel.renal, panel.coag
    advance: BD workup initiated + cath lab booked + fibrinolysis withheld
  6. 6BRANCHING_WORKUP
    Emergent cath with IVUS/OCT to characterize aneurysmal vs vasculitic vs thrombotic lesion morphology + stent landing zones; HLA-B51 typing; pathergy test deferred to outpatient; ophthalmology consult for uveitis assessment; CTA chest/abdomen/pelvis for extra-coronary aneurysms (pulmonary artery aneurysm characteristic, aortic, splenic, renal); MRI/MRA brain if neuro symptoms; rheumatology formal note
    inputs: cor_angio, ivus_or_oct_intracoronary_imaging, hla_b51_typing, pathergy_uveitis_eye_exam_history
    advance: lesion morphology + extra-coronary aneurysm screen + BD activity documented
  7. 7TREATMENT
    Acute reperfusion: PRIMARY PCI ONLY (NO fibrinolysis given aneurysm rupture risk) guided by IVUS/OCT findings; vasculitic / aneurysmal lesions may favor balloon angioplasty + careful stenting in non-aneurysmal landing zones; CABG (arterial conduits preferred — venous grafts may also develop vasculitis) for diffuse multivessel BD vasculitis where PCI infeasible. HIGH-DOSE IMMUNOSUPPRESSION: methylprednisolone 1 g IV × 3 d → prednisone 1 mg/kg PO daily taper; CYCLOPHOSPHAMIDE IV pulse 500-1000 mg/m² (severe / vital-organ vasculo-Behçet — EULAR 2018 PMID 29625968 Class I); INFLIXIMAB / TNF-α inhibitor for refractory or steroid-sparing (Sfikakis 2004 PMID 15571742). Initiate / continue COLCHICINE 0.5-1 mg/d (foundational mucocutaneous + modest vascular benefit). Standard ACS bundle modified: ASA + P2Y12 + UFH; AVOID aspirin monotherapy as sole antithrombotic in pure-vasculitic; AC use cautiously given aneurysm rupture risk (only if clear thrombus + concurrent immunosuppression).
    inputs: sbp, creatinine
    actions: protocol.stemi
    advance: reperfusion (PCI not lytic) + immunosuppression + colchicine initiated + multidisciplinary plan documented
  8. 8DISPOSITION
    CICU 48-72 h post-reperfusion; rheumatology + cardiology + ophthalmology multidisciplinary; cardiothoracic surgery consult if CABG candidate; 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 BD activity assessment (mucocutaneous + ocular + neuro + vascular); steroid taper monitoring (hyperglycemia, infection); echo at 5-7 d for thrombus + LV reassessment + pericardial effusion; serial CTA at 4-6 weeks for aneurysm size + new aneurysm screen
    inputs: echo_post_admission
    actions: panel.cardiac
    advance: inflammation trend + LV function + aneurysm surveillance documented
  10. 10FOLLOWUP
    Lifelong colchicine; maintenance immunosuppression per rheumatology (azathioprine 2-3 mg/kg/d PO OR MMF 1-3 g/d OR TNF-α inhibitor); aggressive secondary prevention (high-intensity statin, BP control, no smoking — smoking is major vasculo-Behçet flare trigger); cardiology + rheumatology + ophthalmology q3 mo first year then q6 mo; surveillance CTA at 3-6 mo then annually for aneurysm; AVOID OCP / estrogen (procoagulant + may flare BD)
    advance: long-term multidisciplinary plan booked + smoking cessation + immunosuppression maintenance scheduled