STEMI — Behçet-disease-associated coronary vasculitis (variable-vessel vasculitis with aneurysms + thrombosis)
Encounter flow
10/12 authoredCanonical 12-phase frame with authored status for this dossier.
Frame
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.
Behçet-associated etiology framed
Patient inputs (12)
Acute-phase reactants — typically elevated in active vasculo-Behçet (unlike pure SLE where CRP often spared); guides immunosuppression intensity
Behçet typical onset 25-30 yr; vasculo-Behçet predominantly young men; STEMI <50 with mucocutaneous features should trigger BD workup
Baseline + contrast nephropathy + cyclophosphamide / MMF dose adjustment + AC dosing
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
STEMI territory localization; pericarditis pattern overlap (BD pericarditis common comorbid)
Quantifies infarct burden; persistent elevation may suggest ongoing vasculitis vs single-event thrombosis
LVEF + regional wall motion + pericardial effusion (BD pericarditis common comorbid) + valve assessment + intracardiac thrombus screen
Hypotension + STEMI in BD with coronary aneurysm thrombosis → cardiogenic shock high probability; rupture of coronary or extra-coronary aneurysm produces shock
Diagnostic + therapeutic gold standard; BD vasculitis often shows aneurysmal coronary segments (saccular), intra-aneurysmal thrombus, multivessel involvement; AVOID fibrinolysis given rupture risk
HLA-B51 frequent (~60% Mediterranean / Silk Road); supports BD diagnosis when clinical features incomplete
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
Pathergy test (sterile pustule at needle prick site) + ophthalmology eye exam (anterior + posterior uveitis, retinal vasculitis) — complete BD criteria assessment
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Severity triggers (7)
- informationallife_threateningbehcet_active_coronary_vasculitis_with_stemiIVUS/OCT confirms vessel-wall inflammation + aneurysm with thrombus + active BD features (mucocutaneous, ocular, vascular, elevated ESR/CRP) at index STEMI → high-dose pulse steroids + cyclophosphamide immediately; AVOID fibrinolysis (rupture risk)Trigger could not be auto-evaluated — needs clinician judgement.
- informationallife_threateningbehcet_pulmonary_artery_aneurysm_with_hemoptysisMassive hemoptysis + pulmonary artery aneurysm on CTA in BD patient → CHARACTERISTIC VASCULO-BEHÇET EMERGENCY; aneurysm rupture imminent or ongoing; mortality without rapid interventionTrigger could not be auto-evaluated — needs clinician judgement.
- informationallife_threateningbehcet_extra_coronary_aneurysm_rupture_with_concurrent_stemiAortic / abdominal / splenic / renal aneurysm rupture with concurrent BD-STEMI → multi-organ vascular emergency; mortality high without simultaneous vascular surgery + cardiac careTrigger could not be auto-evaluated — needs clinician judgement.
- informationallife_threateningbehcet_neuro_behcet_with_concurrent_stemiNew CNS deficits (parenchymal — brainstem common; or vascular — cerebral venous thrombosis) + STEMI in BD patient → concurrent neuro-Behçet flare; immunosuppression must address both compartmentsTrigger could not be auto-evaluated — needs clinician judgement.
- informationallife_threateningbehcet_sudden_cardiac_death_risk_after_eventEF <35 + non-sustained VT on telemetry post-BD-STEMI → SCD risk in 40-90 d window (often young patients with lifetime ICD implications)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalsevereimmunosuppression_related_infection_during_taperNew fever / infiltrate / cytopenia during high-dose steroid + cyclophosphamide / TNF-α inhibitor — opportunistic infection (PJP, CMV, fungal, TB reactivation especially with TNF-α) high riskTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverebehcet_smoking_relapse_with_vascular_flareSmoking relapse + new vascular event (DVT, arterial thrombosis, aneurysm enlargement) in BD patient — smoking is major vasculo-Behçet flare triggerTrigger could not be auto-evaluated — needs clinician judgement.
Workflow calculators
Run this disease's risk and dosing calculators inline.
Recommended regimen
Behçet coronary vasculitis + STEMI regimen — combines acute reperfusion (PCI ONLY, NO lysis) with high-dose immunosuppression and lifelong colchicine + maintenance immunosuppression + secondary prevention- aspirinfirst lineantiplatelet_cox1162-325 mg chewed load → 81 mg daily lifelong • PO • daily indefinitelytriggers: behcet_stemi_confirmed_with_thrombusAHA 2025 ACS Class I + EULAR 2018 (PMID 29625968) — ASA in BD arterial event with thrombus; AVOID aspirin MONOTHERAPY as sole antithrombotic in pure-vasculitic phenotype (does not address inflammation)rxcui 243670
- ticagrelorfirst linep2y12_inhibitor180 mg load → 90 mg BID • PO • BID × 12 mo standard DAPT, then reassess given vasculitis statustriggers: behcet_stemi_pci_plannedPLATO PMID 19717846; standard ACS DAPT applies; duration extension if persistent vasculitis activity or recurrent thrombusrxcui 1116632
- unfractionated_heparinfirst lineparenteral_anticoagulant70-100 U/kg IV bolus + activated infusion • IV • bolus + infusion at PCItriggers: behcet_stemi_pci_plannedAHA 2025 Class I for PCI; UFH preferred over LMWH given more reversible if aneurysm bleeding complicationrxcui 5224
- atorvastatinfirst linestatin_high_intensity80 mg daily lifelong • PO • dailytriggers: behcet_stemi_confirmedPROVE-IT PMID 15007110; high-intensity statin lifelong post-MI; pleiotropic anti-inflammatory effect particularly relevant for BD endothelial dysfunctionrxcui 83367
- colchicinefirst lineantimitotic_microtubule_inhibitor0.5-1 mg PO daily (Mediterranean fever dose); 0.6 mg BID alternative • PO • daily lifelongtriggers: behcet_diagnosis_confirmed, behcet_stemi_index_eventEULAR 2018 (PMID 29625968) Class I — colchicine foundational for ALL BD patients; mucocutaneous + arthritis benefit + modest vascular benefit; renal-adjusted; eGFR <30 contraindicated for chronic userxcui 2683
- methylprednisolonerescuecorticosteroid_iv_pulse1 g IV daily × 3 days • IV • pulse × 3 dtriggers: confirmed_behcet_coronary_vasculitis_via_ivus_oct_or_active_clinical_features, severe_vasculo_behcet_flareEULAR 2018 (PMID 29625968) Class I — pulse steroids for severe / vital-organ vasculo-Behçet including coronary vasculitis; bridge to oral prednisone + steroid-sparing agentrxcui 6902
- prednisonefirst linecorticosteroid_oral1 mg/kg PO daily, taper over months • PO • daily with taper scheduletriggers: post_pulse_steroid_taper, maintenance_behcet_immunosuppressionEULAR 2018 (PMID 29625968) — oral steroid taper after pulse; goal is steroid minimization with steroid-sparing agent (azathioprine / MMF / TNF-α inhibitor)rxcui 8640
- cyclophosphamideadd oncytotoxic_alkylating500-1000 mg/m² IV monthly × 6 (or low-dose Euro-Lupus 500 mg q2w × 6) • IV • monthly × 6triggers: severe_vasculo_behcet_with_coronary_aneurysm_or_pulmonary_artery_aneurysm, refractory_to_steroids_aloneEULAR 2018 (PMID 29625968) Class I — cyclophosphamide for severe / vital-organ vasculo-Behçet; coronary + pulmonary artery aneurysms qualify; gonadotoxic — counseling requiredrxcui 3002
- infliximabsecond linetnf_alpha_inhibitor_monoclonal5 mg/kg IV at 0, 2, 6 weeks then q6-8 weeks • IV • q6-8 weekstriggers: refractory_behcet_coronary_vasculitis_despite_steroids_+_cyclophosphamide, steroid_sparing_alternative, concurrent_uveitisEULAR 2018 (PMID 29625968) — TNF-α inhibitor (infliximab or adalimumab) for refractory severe BD; Sfikakis 2004 (PMID 15571742) supports use; screen for latent TB + hepatitis pre-initiationrxcui 191831
- azathioprineadd onimmunosuppressant_purine_synthesis_inhibitor2-3 mg/kg/d PO; check TPMT before initiation • PO • dailytriggers: steroid_sparing_maintenance, eye_disease_preventionHamuryudan 2004 (PMID 14730108) — azathioprine reduces eye disease + vascular events in BD; foundational maintenance steroid-sparing agentrxcui 1256
- mycophenolate_mofetilsecond lineimmunosuppressant_imp_dh_inhibitor1-3 g/d divided BID • PO • BIDtriggers: azathioprine_intolerant_or_TPMT_deficient, maintenance_immunosuppressionEULAR 2018 — MMF alternative steroid-sparing maintenance; teratogenic — switch off before conceptionrxcui 68149
- warfarincomorbidity specificvitamin_k_antagonist5 mg daily; INR target 2-3 ONLY if clear thrombus burden + concurrent immunosuppression; AVOID in pure-vasculitic without thrombus given aneurysm rupture risk • PO • dailytriggers: concurrent_dvt_pe_with_documented_thrombus_+_active_immunosuppression, mechanical_valve_indication_onlyEULAR 2018 (PMID 29625968) — AC controversial in BD; immunosuppression is primary therapy for vasculo-Behçet vascular events; AC ONLY with concurrent immunosuppression and clear thrombus; aneurysm rupture risk; venous thrombosis treated with immunosuppression rather than AC alone in BDrxcui 11289
- carvedilolfirst linebeta_blocker_nonselective3.125 mg BID titrate • PO • BIDtriggers: behcet_stemi_with_lv_dysfunction, ef_below_40CAPRICORN PMID 11356436 — post-MI BB benefit; carvedilol preferred for HFrEF GDMTrxcui 20352
- sacubitril-valsartanadd onarni24/26 mg BID titrate • PO • BIDtriggers: behcet_stemi_with_ef_below_40_post_eventPIONEER-HF PMID 30403955; ACC/AHA 2022 HF Class I if HFrEF persists post-MIrxcui 1656328
outpatient playbook — drug actions (1)
- 1. continue lifelong bundlerxcui 243670ASA 81 + atorvastatin 80 + colchicine 0.5-1 mg daily + maintenance immunosuppression (azathioprine / MMF / TNF-α inhibitor) per rheumatology + GDMT if HFrEF • PO • as scheduledtrigger: BD coronary disease lifelongEULAR 2018 + AHA 2025 ACS
Auto-drafted A&P note
outpatientSubjective
- Possible entry pathways: STEMI in patient with established Behçet disease — coronary vasculitis vs aneurysm thrombosis vs spasm triage; STEMI in young man (20-40 yr; Mediterranean / Silk Road origin) with recurrent oral + genital ulcers + uveitis + skin lesions → de novo Behçet consideration; Coronary cath shows aneurysmal coronary segments (saccular, mycotic-like appearance) with intra-aneurysmal thrombus → variable-vessel vasculitis suspect; AVOID fibrinolysis given rupture risk.
Objective
- No vitals, labs, or imaging entered for this encounter.
Assessment
**STEMI — Behçet-disease-associated coronary vasculitis (variable-vessel vasculitis with aneurysms + thrombosis)** (cardio.stemi.behcet-coronary-vasculitis.v1). Scope: 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. No severity triggers fired against current inputs.
Plan
Regimen axis: **Behçet coronary vasculitis + STEMI regimen — combines acute reperfusion (PCI ONLY, NO lysis) with high-dose immunosuppression and lifelong colchicine + maintenance immunosuppression + secondary prevention**. 1. aspirin 162-325 mg chewed load → 81 mg daily lifelong PO daily indefinitely (antiplatelet_cox1, first line) — AHA 2025 ACS Class I + EULAR 2018 (PMID 29625968) — ASA in BD arterial event with thrombus; AVOID aspirin MONOTHERAPY as sole antithrombotic in pure-vasculitic phenotype (does not address inflammation) 2. ticagrelor 180 mg load → 90 mg BID PO BID × 12 mo standard DAPT, then reassess given vasculitis status (p2y12_inhibitor, first line) — PLATO PMID 19717846; standard ACS DAPT applies; duration extension if persistent vasculitis activity or recurrent thrombus 3. unfractionated_heparin 70-100 U/kg IV bolus + activated infusion IV bolus + infusion at PCI (parenteral_anticoagulant, first line) — AHA 2025 Class I for PCI; UFH preferred over LMWH given more reversible if aneurysm bleeding complication 4. atorvastatin 80 mg daily lifelong PO daily (statin_high_intensity, first line) — PROVE-IT PMID 15007110; high-intensity statin lifelong post-MI; pleiotropic anti-inflammatory effect particularly relevant for BD endothelial dysfunction 5. colchicine 0.5-1 mg PO daily (Mediterranean fever dose); 0.6 mg BID alternative PO daily lifelong (antimitotic_microtubule_inhibitor, first line) — EULAR 2018 (PMID 29625968) Class I — colchicine foundational for ALL BD patients; mucocutaneous + arthritis benefit + modest vascular benefit; renal-adjusted; eGFR <30 contraindicated for chronic use 6. methylprednisolone 1 g IV daily × 3 days IV pulse × 3 d (corticosteroid_iv_pulse, rescue) — EULAR 2018 (PMID 29625968) Class I — pulse steroids for severe / vital-organ vasculo-Behçet including coronary vasculitis; bridge to oral prednisone + steroid-sparing agent 7. prednisone 1 mg/kg PO daily, taper over months PO daily with taper schedule (corticosteroid_oral, first line) — EULAR 2018 (PMID 29625968) — oral steroid taper after pulse; goal is steroid minimization with steroid-sparing agent (azathioprine / MMF / TNF-α inhibitor) 8. cyclophosphamide 500-1000 mg/m² IV monthly × 6 (or low-dose Euro-Lupus 500 mg q2w × 6) IV monthly × 6 (cytotoxic_alkylating, add on) — EULAR 2018 (PMID 29625968) Class I — cyclophosphamide for severe / vital-organ vasculo-Behçet; coronary + pulmonary artery aneurysms qualify; gonadotoxic — counseling required 9. infliximab 5 mg/kg IV at 0, 2, 6 weeks then q6-8 weeks IV q6-8 weeks (tnf_alpha_inhibitor_monoclonal, second line) — EULAR 2018 (PMID 29625968) — TNF-α inhibitor (infliximab or adalimumab) for refractory severe BD; Sfikakis 2004 (PMID 15571742) supports use; screen for latent TB + hepatitis pre-initiation 10. azathioprine 2-3 mg/kg/d PO; check TPMT before initiation PO daily (immunosuppressant_purine_synthesis_inhibitor, add on) — Hamuryudan 2004 (PMID 14730108) — azathioprine reduces eye disease + vascular events in BD; foundational maintenance steroid-sparing agent 11. mycophenolate_mofetil 1-3 g/d divided BID PO BID (immunosuppressant_imp_dh_inhibitor, second line) — EULAR 2018 — MMF alternative steroid-sparing maintenance; teratogenic — switch off before conception 12. warfarin 5 mg daily; INR target 2-3 ONLY if clear thrombus burden + concurrent immunosuppression; AVOID in pure-vasculitic without thrombus given aneurysm rupture risk PO daily (vitamin_k_antagonist, comorbidity specific) — EULAR 2018 (PMID 29625968) — AC controversial in BD; immunosuppression is primary therapy for vasculo-Behçet vascular events; AC ONLY with concurrent immunosuppression and clear thrombus; aneurysm rupture risk; venous thrombosis treated with immunosuppression rather than AC alone in BD 13. carvedilol 3.125 mg BID titrate PO BID (beta_blocker_nonselective, first line) — CAPRICORN PMID 11356436 — post-MI BB benefit; carvedilol preferred for HFrEF GDMT 14. sacubitril-valsartan 24/26 mg BID titrate PO BID (arni, add on) — PIONEER-HF PMID 30403955; ACC/AHA 2022 HF Class I if HFrEF persists post-MI Setting playbook (outpatient) — Lifelong cardiology + rheumatology + ophthalmology multidisciplinary surveillance: serial CTA every 6-12 mo for coronary + extra-coronary aneurysms; lifelong colchicine + maintenance immunosuppression; continued GDMT if HFrEF; aggressive secondary prevention (BP <130/80, lipid LDL <55 per ESC vasculitis high-risk, smoking cessation maintained); pregnancy + family planning counseling (colchicine compatible with pregnancy with rheum input; cyclophosphamide / MMF teratogenic — switch to azathioprine before conception) 15. continue lifelong bundle ASA 81 + atorvastatin 80 + colchicine 0.5-1 mg daily + maintenance immunosuppression (azathioprine / MMF / TNF-α inhibitor) per rheumatology + GDMT if HFrEF PO as scheduled — BD coronary disease lifelong (EULAR 2018 + AHA 2025 ACS) Non-pharmacologic actions: - Pregnancy counseling: colchicine compatible (low-dose); cyclophosphamide + MMF TERATOGENIC — switch to azathioprine ≥3 mo before conception; AVOID estrogen contraception - Lifelong cardiac rehab maintenance + tailored exercise - Mental health continuity (chronic disease + young adult adjustment) - Annual influenza + 5-yr pneumococcal vaccinations (avoid live vaccines) - Smoking cessation maintained — major vasculo-Behçet flare trigger - Sun protection counseling AVOID / contraindication checks: - AVOID_fibrinolysis_in_behcet_stemi_due_to_coronary_aneurysm_rupture_risk — EULAR 2018 (PMID 29625968) - AVOID_aspirin_monotherapy_as_sole_antithrombotic_in_pure_vasculitic_phenotype_does_not_address_inflammation - AC_use_cautiously_in_BD_aneurysm_rupture_risk_use_only_with_concurrent_immunosuppression — EULAR 2018 - Colchicine_renal_dose_adjustment_avoid_chronic_use_eGFR_below_30 - Cyclophosphamide_gonadotoxicity_counseling_+_fertility_preservation - MMF_teratogenic_switch_off_before_conception (FDA category D) - Cyclophosphamide_teratogenic (FDA category D) - Steroid_taper_must_account_for_adrenal_insufficiency_after_>3wk_use - TNF_alpha_inhibitor_screen_latent_TB_+_hepatitis_pre_initiation - AVOID_estrogen_OCP_HRT_in_BD (procoagulant + may flare disease) - Smoking_cessation_critical_major_vasculo_Behçet_flare_trigger - Live_vaccines_contraindicated_on_immunosuppression
Monitoring
Regimen monitoring: - echo at 5-7d post event for thrombus screen + lv function + pericardial effusion - serial troponin for persistent elevation indicating ongoing vasculitis - serial CTA at 4-6 weeks then 3-6 months then annually for coronary + extra-coronary aneurysm surveillance - ESR CRP at baseline then q1-3mo for vasculitis activity - CBC LFTs creatinine q2-4 weeks during immunosuppression - TPMT pre azathioprine - TB quantiferon + hepatitis panel pre TNF alpha inhibitor - pneumocystis jirovecii prophylaxis during high-dose immunosuppression - shingrix + pneumococcal vaccinations pre immunosuppression if possible - ophthalmology q6mo for uveitis surveillance Setting (outpatient) monitoring: - Coronary + extra-coronary CTA every 6-12 mo - Annual lipid + BP + CBC + LFTs + creatinine + ESR + CRP - BD activity assessment quarterly - Annual eye exam (ophthalmology) Follow-up plan: 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) - Close-out criterion: long-term multidisciplinary plan booked + smoking cessation + immunosuppression maintenance scheduled Monitoring phase: 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
Disposition
Current setting: outpatient — Lifelong cardiology + rheumatology + ophthalmology multidisciplinary surveillance: serial CTA every 6-12 mo for coronary + extra-coronary aneurysms; lifelong colchicine + maintenance immunosuppression; continued GDMT if HFrEF; aggressive secondary prevention (BP <130/80, lipid LDL <55 per ESC vasculitis high-risk, smoking cessation maintained); pregnancy + family planning counseling (colchicine compatible with pregnancy with rheum input; cyclophosphamide / MMF teratogenic — switch to azathioprine before conception) Disposition criteria: - Long-term continuation; cross-link to cardio.hf.core.v1 if HFrEF persists; cross-link to vasc.large-vessel-vasculitis.* if extra-coronary involvement predominant Escalation triggers (move to higher acuity): - New ACS or recurrent CAD on CTA → cardiology + rheumatology — assess vasculitis activity vs atherosclerosis - New or growing extra-coronary aneurysm → vascular surgery + escalate immunosuppression - Pulmonary artery aneurysm with hemoptysis → emergent thoracic surgery + escalate immunosuppression - EF declining despite GDMT → advanced HF / transplant evaluation - Bleeding on DAPT ± AC → reassess regimen - BD flare → rheumatology — escalate immunosuppression - Pregnancy planned → switch teratogenic meds 3 mo prior + high-risk obstetrics + cardiology team - Smoking relapse → enhanced cessation support — major vasculo-Behçet flare risk
Earlier-Return Triggers
Return-precaution thresholds (watch for): - [LIFE_THREATENING] IVUS/OCT confirms vessel-wall inflammation + aneurysm with thrombus + active BD features (mucocutaneous, ocular, vascular, elevated ESR/CRP) at index STEMI → high-dose pulse steroids + cyclophosphamide immediately; AVOID fibrinolysis (rupture risk) - [LIFE_THREATENING] Massive hemoptysis + pulmonary artery aneurysm on CTA in BD patient → CHARACTERISTIC VASCULO-BEHÇET EMERGENCY; aneurysm rupture imminent or ongoing; mortality without rapid intervention - [LIFE_THREATENING] Aortic / abdominal / splenic / renal aneurysm rupture with concurrent BD-STEMI → multi-organ vascular emergency; mortality high without simultaneous vascular surgery + cardiac care
Citations
- 2025 ACC/AHA ACS Guideline + EULAR 2018 Behçet syndrome management recommendations (Hatemi ARD 2018 PMID 29625968) + International Study Group BD criteria (Lancet 1990 PMID 1970380) + Chapel Hill Consensus 2012 vasculitis nomenclature (PMID 23045170) [PMID:29625968](https://pubmed.ncbi.nlm.nih.gov/29625968/) - Cited evidence (PMID 37622670) [PMID:37622670](https://pubmed.ncbi.nlm.nih.gov/37622670/) - Cited evidence (PMID 23045170) [PMID:23045170](https://pubmed.ncbi.nlm.nih.gov/23045170/) - Cited evidence (PMID 1970380) [PMID:1970380](https://pubmed.ncbi.nlm.nih.gov/1970380/) - Cited evidence (PMID 22588748) [PMID:22588748](https://pubmed.ncbi.nlm.nih.gov/22588748/) Last reconciled with current guidelines: 2026-05-15.
- 2025 ACC/AHA ACS Guideline + EULAR 2018 Behçet syndrome management recommendations (Hatemi ARD 2018 PMID 29625968) + International Study Group BD criteria (Lancet 1990 PMID 1970380) + Chapel Hill Consensus 2012 vasculitis nomenclature (PMID 23045170) — PMID:29625968
- Cited evidence (PMID 37622670) — PMID:37622670
- Cited evidence (PMID 23045170) — PMID:23045170
- Cited evidence (PMID 1970380) — PMID:1970380
- Cited evidence (PMID 22588748) — PMID:22588748