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cardio.dvt.behcet-disease.v1

DVT/VTE in Behçet's disease (variable-vessel vasculitis)

cardiologyacuteadultacuteinpatienttransitionoutpatient
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Phase E variant of cardio.dvt.core.v1 — narrowed to DVT/VTE complicating Behçet's disease (variable-vessel vasculitis with venous predominance). Inherits diagnostic arc and DOAC chronic regimen from parent via routing; specializes for the combined AC + immunosuppression treatment paradigm, the strict pulmonary-aneurysm screening BEFORE AC, and multidisciplinary rheumatology + ophthalmology + cardiology care. Manifest pointer reuses cardio.dvt.core.v1 manifest. Design-brief pointer reuses parent (Behçet-specific differences documented inline). Distinguishing features vs generic DVT: (1) AC alone is INSUFFICIENT — recurrence ≈40% at 2 y without immunosuppression; (2) pulmonary artery aneurysm is a strict AC contraindication and MUST be excluded with CT-angio chest before starting AC in any Behçet patient with hemoptysis/dyspnea; (3) cerebral venous sinus thrombosis and vena cava syndromes are common and require cyclophosphamide induction in addition to AC; (4) treatment hierarchy follows EULAR 2018 (azathioprine + steroids first-line; cyclophosphamide for severe vascular disease; TNF-α inhibitor — infliximab/adalimumab — for refractory or sight-threatening); (5) duration of AC is indefinite while disease activity persists and is tapered as remission is achieved. Status INTEGRATED. Authored 2026-05-15 by shard-06-cardio-acute as Behçet vascular DVT variant.

Entry points (4)

  • symptom
    Unilateral leg swelling with proven DVT in patient with recurrent oral aphthae + genital ulcers ± uveitis — pretest probability for vascular Behçet is high (ISG / ICBD criteria)
    unilateral_leg_swelling_with_oral_genital_ulcers
  • history
    VTE in young (under 40) male of Turkish, Iranian, Mediterranean, or Central/East Asian descent — prompts Behçet workup; HLA-B51 association
    vte_in_young_male_silk_road_origin
  • history
    Recurrent DVT with elevated CRP/ESR + ocular inflammation or skin pathergy — vasculitic VTE should be considered
    recurrent_dvt_with_systemic_inflammation
  • imaging
    Cerebral venous sinus thrombosis on MR-venography in young patient with mucocutaneous ulcers — Behçet CVST workup pathway
    cerebral_venous_sinus_thrombosis_with_systemic_features

Required inputs (14)

  • agerequired
    demographic • used at CONTEXT
    Behçet typically presents in 3rd-4th decade; VTE risk concentrates in young adult males — informs pretest probability
  • sexrequired
    demographic • used at CONTEXT
    Male sex confers higher vascular and ocular morbidity; informs prognosis and immunosuppression aggressiveness
  • silk_road_ancestryrequired
    history • used at CONTEXT
    Turkish, Iranian, Mediterranean, Central/East Asian ancestry — HLA-B51 enriched populations; pretest probability for Behçet rises
  • recurrent_oral_ulcersrequired
    history • used at CONTEXT
    Major ISG / ICBD criterion — required for diagnosis; documents disease activity
  • genital_ulcer_historyrequired
    history • used at CONTEXT
    Major ISG / ICBD criterion; informs diagnosis and disease activity scoring
  • ocular_inflammation_uveitisrequired
    history • used at CONTEXT
    Sight-threatening posterior uveitis is a major Behçet complication and an independent indication for immunosuppression intensification
  • leg_swellingrequired
    symptom • used at ENTRY
    Cardinal symptom of proximal DVT
  • compression_usrequired
    imaging • used at INITIAL_WORKUP
    Initial confirmation of DVT location (proximal vs distal vs vena cava extension)
  • cta_chest_for_pulmonary_aneurysmrequired
    imaging • used at RED_FLAGS
    CT-angio chest BEFORE anticoagulation in any Behçet patient with hemoptysis or unexplained dyspnea — pulmonary artery aneurysm is a strict AC contraindication and must be excluded
  • crp_esrrequired
    lab • used at CONTEXT
    Inflammatory markers tracking disease activity; target normalisation parallels AC tapering decision
  • creatininerequired
    lab • used at TREATMENT
    eGFR for DOAC dosing and azathioprine/cyclophosphamide dose adjustment
  • cbcrequired
    lab • used at INITIAL_WORKUP
    Baseline platelet for AC initiation; ongoing surveillance during azathioprine and cyclophosphamide therapy
  • hla_b51
    lab • used at BRANCHING_WORKUP
    HLA-B51 supports diagnosis (~60% of patients positive in endemic populations); not pathognomonic
  • bleed_riskrequired
    history • used at RED_FLAGS
    HAS-BLED + falls + GI bleed history drives indefinite-AC eligibility and informs anticoagulant intensity

12-phase flow (11)

  1. 1FRAME
    Behçet vascular phenotype: variable-vessel vasculitis with venous predominance; VTE driven by vessel-wall inflammation, NOT a primary coagulation defect; AC alone is insufficient — recurrence ≈40% at 2 y without immunosuppression vs much lower with azathioprine ± steroids per EULAR 2018
    inputs: leg_swelling
    advance: vasculitic etiology framed
  2. 2ENTRY
    Wells DVT score + compression US; document mucocutaneous ulcer history + ocular involvement + family/ancestry history; pretest probability for Behçet
    inputs: recurrent_oral_ulcers, silk_road_ancestry
    advance: pretest probability + Behçet workup indication documented
  3. 3CONTEXT
    Age, sex, ancestry, ulcer history, ocular inflammation, prior VTE sites (look for cerebral venous sinus, vena cava, hepatic vein), pathergy test (positive in endemic populations), HLA-B51
    inputs: age, sex, genital_ulcer_history, ocular_inflammation_uveitis, crp_esr
    advance: context complete
  4. 4RED_FLAGS
    CT-angio chest BEFORE AC if any hemoptysis or unexplained dyspnea — pulmonary artery aneurysm is a strict AC contraindication; concurrent cerebral venous sinus thrombosis (MR-venography); concurrent vena cava syndromes; vasculitis flare with sight-threatening uveitis or CNS involvement; absolute AC contraindication; pregnancy
    inputs: cta_chest_for_pulmonary_aneurysm, bleed_risk
    actions: pe_full, thrombocytopenia
    advance: pulmonary aneurysm excluded + critical features screened
  5. 5INITIAL_WORKUP
    Compression US (proximal vs distal vs vena cava extension); CBC + BMP + INR/PTT + CRP + ESR; D-dimer if pretest probability borderline; troponin + BNP if PE confirmed
    inputs: compression_us, cbc, creatinine
    actions: panel.cardiac, panel.renal
    advance: imaging confirms DVT and pulmonary aneurysm excluded
  6. 6BRANCHING_WORKUP
    Behçet diagnostic confirmation: ISG (1990) and ICBD (2014) criteria; ophthalmology slit-lamp for posterior uveitis; HLA-B51 (supportive, not pathognomonic); MR-venography if neuro symptoms (CVST exclusion); abdominal cross-sectional imaging if Budd-Chiari suspected; pathergy test in endemic populations
    inputs: hla_b51
    advance: Behçet diagnostic criteria + extra-vascular manifestations documented
  7. 7RISK_STRATIFICATION
    Wells DVT, HAS-BLED, eGFR; Behçet disease activity (BDCAF / disease-activity index); presence of high-risk vascular sites (vena cava, cerebral venous sinus, hepatic) shifts toward more aggressive immunosuppression (cyclophosphamide rather than azathioprine first-line)
    inputs: bleed_risk
    actions: calc.wells_dvt, calc.has_bled
    advance: AC duration + immunosuppression plan documented with rationale
  8. 8TREATMENT
    Co-administered AC + immunosuppression. AC: DOAC (apixaban or rivaroxaban) for routine peripheral DVT — case series support; LMWH bridge for inpatient; warfarin if APS coexists or frequent dose-interruption needs; AVOID AC if pulmonary artery aneurysm. Immunosuppression: azathioprine + prednisone first-line (EULAR 2018 Class I); cyclophosphamide for severe / vena cava / CVST disease; INFLIXIMAB or ADALIMUMAB for refractory or sight-threatening; colchicine for mucocutaneous baseline. Duration: AC indefinite while active; can taper as remission achieved
    inputs: creatinine, bleed_risk
    advance: acute AC + immunosuppression bundle started
  9. 9DISPOSITION
    Outpatient for uncomplicated peripheral DVT with rheumatology co-management; admit if CVST, vena cava extension, suspected pulmonary aneurysm, sight-threatening uveitis, or social barriers
    advance: disposition + multidisciplinary handoff documented
  10. 10MONITORING
    CBC + LFT + creatinine at 2 weeks then monthly during azathioprine titration (TPMT screen ideal at start); CRP/ESR monthly to track disease activity; ophthalmology follow-up; bleed surveillance; PTS Villalta at 3/6/12 mo; cyclophosphamide cumulative-dose tracking with bladder cancer screen
    actions: panel.cardiac
    advance: monitoring schedule documented
  11. 11FOLLOWUP
    Long-term rheumatology + cardiology + ophthalmology multidisciplinary care; AC continuation reviewed annually with disease activity; immunosuppression taper as remission achieved; family planning counseling (azathioprine compatible with pregnancy, cyclophosphamide is gonadotoxic — preserve fertility before treatment); vaccinations updated before biologics
    advance: multidisciplinary maintenance plan + reproductive counseling documented