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Patient handout

Antiphospholipid syndrome (APS) DVT — warfarin preferred, DOAC avoided

PRODUCTION

1. Your condition

This handout is for antiphospholipid syndrome (aps) dvt — warfarin preferred, doac avoided. Your care team identified this based on: unilateral leg swelling/pain with prior aps diagnosis or strong aps suspicion (recurrent vte, arterial + venous events, pregnancy morbidity, autoimmune disease — particularly sle).

Other reasons your team may use this plan: unprovoked vte in patient with ≥3 consecutive miscarriages <10 wk or ≥1 fetal death ≥10 wk or severe preeclampsia/placental insufficiency — sapporo/sydney pregnancy-morbidity criteria; patient with both prior arterial event (stroke, mi, tia) and venous event — aps until proven otherwise; unexplained aptt prolongation that does not correct with mixing study — lupus anticoagulant suspected.

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
warfarin5 mg PO daily; target INR 2-3 (first venous APS event), 2.5-3.5 (arterial APS or recurrent venous APS), 3-4 (selected triple-positive cases per expert opinion)POdaily lifelongTRAPS (Pengo Blood 2018 PMID 30196097) — warfarin SUPERIOR to rivaroxaban in triple-positive APS; EULAR 2019 — warfarin first-line; lifelong AC per APS clinical criterion
enoxaparin1 mg/kg SC BID (reduce to 1 mg/kg SC daily if CrCl <30); overlap with warfarin until INR therapeutic for ≥2 consecutive daysSCBIDASH 2020 (PMID 33007077); ASH 2018 pregnancy (PMID 30482767) — LMWH first-line in pregnancy; standard initial AC overlap with warfarin (≥5 d + INR therapeutic ≥2 d)
aspirin81 mg PO dailyPOdailyEULAR 2019 — low-dose ASA add-on for arterial APS; antepartum low-dose ASA + LMWH for APS pregnancy improves fetal outcomes; potential benefit in recurrent thrombosis on therapeutic warfarin
hydroxychloroquine200-400 mg PO daily (max 5 mg/kg/day actual body weight per AAO retinopathy guidance)POdailyEULAR 2019 — hydroxychloroquine adjunct in SLE-associated APS reduces thrombosis recurrence; observational data support adjunct in recurrent APS thrombosis on warfarin
heparin80 U/kg IV bolus + 18 U/kg/h targeting aPTT 1.5-2.5× (LA may falsely prolong aPTT — use anti-Xa assay if available)IVcontinuousReversibility for peri-procedural management; anti-Xa monitoring preferred when LA confounds aPTT (ACCP 2021)
AVOID rivaroxabanAVOIDN/AN/ATRAPS (Pengo Blood 2018 PMID 30196097) — trial terminated early for arterial harm in triple-positive APS; ISTH 2020 + EULAR 2019 advise against rivaroxaban in APS
AVOID apixaban in APSAVOIDN/AN/AASTRO-APS apixaban arm (Woller Blood Adv 2022) — terminated for harm in arterial APS; class effect concern; EULAR 2019
methylprednisoloneCAPS: methylprednisolone 1 g IV daily × 3 d → taperIVdailyCAPS Registry — corticosteroids one of the four pillars (anticoagulation + corticosteroids + plasma exchange + IVIG ± rituximab/eculizumab) in CAPS; mortality benefit in observational data
fentanyl25-100 µg IV q5-10 min titrateIVPRNPain control; CAPS multi-organ ischemia is severely painful; reduces sympathetic drive

Plan: Antiphospholipid syndrome anticoagulation — WARFARIN preferred (INR target by phenotype), LMWH in pregnancy, DOAC INFERIOR and avoided (TRAPS, ASTRO-APS, EULAR 2019)

3. When to call your provider

Contact your care team if any of the following happen:

  • Recurrent VTE on therapeutic INR → escalate intensity; consider hydroxychloroquine adjunct or LMWH switch
  • Pregnancy → switch to LMWH + ASA
  • Major bleed → reverse, hold, reassess AC intensity (rarely stop)
  • CAPS features → ED + ICU multi-modality

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • CAPS — ≥3 organ thromboses ≤1 week with histopathological confirmation of small-vessel thrombosis (or strong clinical surrogate); life-threatening with ~37% mortality even with treatment(life-threatening)
  • Patient with established APS diagnosis found to be on rivaroxaban, apixaban, edoxaban, or dabigatran — must transition to warfarin immediately given TRAPS / ASTRO-APS evidence of harm
  • Triple-positive APS woman planning pregnancy — highest fetal/maternal risk requires pre-conception planning with LMWH + low-dose ASA antepartum + 6-wk postpartum LMWH; warfarin discontinuation at conception (teratogenic in 1st trimester)
  • New thrombotic event in APS patient with documented therapeutic INR 2-3 — must escalate intensity (INR 2.5-3.5 or 3-4) or add adjunct (low-dose ASA, hydroxychloroquine, or LMWH overlap)

5. Follow-up

Hematology + rheumatology co-management; reproductive planning for women (LMWH + low-dose ASA antepartum + 6-wk postpartum; hydroxychloroquine adjunct); estrogen avoidance lifelong; cardiovascular risk modification; APS patient card for emergency providers; no DOAC prescription warning in EHR

6. Sources

Guideline: EULAR 2019 APS management + ASH 2020 VTE Treatment + ASH 2018 thrombophilia testing + ASH 2018 VTE in Pregnancy + ACCP/CHEST 2021 + 2023 ACR/EULAR APS classification criteria

  1. pubmed.ncbi.nlm.nih.gov/30196097
  2. pubmed.ncbi.nlm.nih.gov/27932287
  3. pubmed.ncbi.nlm.nih.gov/29562136