| Clinical Criteria (โฅ 1) | Lab Criteria (โฅ 1, confirmed ร 2) |
|---|---|
| Vascular thrombosis: arterial, venous, or small vessel thrombosis in any organ (DVT, PE, stroke, MI, renal/hepatic thrombosis) | Lupus anticoagulant (LA) -most strongly associated with thrombosis |
| Pregnancy morbidity: โฅ 1 unexplained fetal death โฅ 10 weeks, โฅ 3 unexplained consecutive losses < 10 weeks, premature birth < 34 weeks due to eclampsia/placental insufficiency | Anticardiolipin (aCL) IgG or IgM -medium-high titer |
| Anti-ฮฒ2 glycoprotein I IgG or IgM |
| Scenario | Treatment |
|---|---|
| Venous thrombosis (DVT/PE) | Warfarin INR 2โ3, indefinite. No DOACs. |
| Arterial thrombosis (stroke, MI) | Warfarin INR 2โ3 (some advocate INR 3โ4 for arterial events, controversial). ยฑ aspirin. |
| Obstetric APS (no thrombosis) | LMWH (enoxaparin prophylactic dose) + low-dose aspirin throughout pregnancy. |
| Catastrophic APS (CAPS) | Triple therapy: anticoagulation + high-dose steroids + plasma exchange or IVIG. Mortality ~50%. Multiorgan thrombotic microangiopathy. |
Patient: 34-year-old man with known triple-positive APS on warfarin, presenting with new left leg swelling. INR 1.4 (subtherapeutic, missed doses). Compression US confirms acute proximal DVT.
Key findings: Triple-positive: LA+, aCL IgG 82 GPL, anti-ฮฒ2GP1 IgG+. Prior DVT 2 years ago. No SLE features.
Management:
Teaching point: Triple-positive APS has the highest thrombotic risk. DOACs are absolutely contraindicated. Warfarin adherence is critical, even brief subtherapeutic periods can lead to recurrent events.
Patient: 28-year-old woman with known APS (single-positive aCL), hospitalized for pneumonia. Over 3 days develops acute renal failure (Cr 1.0 โ 4.2), altered mental status, thrombocytopenia (platelets 38K), and livedo reticularis with digital ischemia.
Key findings: Schistocytes on smear. LDH 1,200. Multi-organ thrombosis on imaging: renal infarcts, cerebral microthrombi on MRI. Infection triggered CAPS.
Management:
Teaching point: CAPS = multi-organ thrombosis developing in less than 1 week. Most often triggered by infection, surgery, or anticoagulation withdrawal. Recognize by the pattern: โฅ 3 organ systems + microvascular thrombosis + known aPL.
Patient: 31-year-old woman with 3 consecutive first-trimester miscarriages. No prior thrombosis. Workup reveals persistent anticardiolipin IgG (52 GPL) and anti-ฮฒ2GP1 IgG positive on two occasions 14 weeks apart. LA negative.
Key findings: Meets criteria for obstetric APS: โฅ 3 consecutive pregnancy losses before 10 weeks + persistent aPL positivity (confirmed at โฅ 12 weeks).
Management:
Teaching point: Obstetric APS requires persistent aPL positivity on 2 tests โฅ 12 weeks apart to avoid overdiagnosis from transient infection-related positivity. ASA + LMWH is the standard regimen. Warfarin is teratogenic, must switch to LMWH.
| Parameter | Frequency | Target / Action |
|---|---|---|
| Vitals | q4h floor, q1โ2h ICU | HR, BP, RR, SpOโ, Temp -notify for significant deviations |
| Labs (BMP, CBC) | Daily AM or as indicated | Trend Cr, Kโบ, WBC, Hgb -adjust treatment based on trajectory |
| Disease-specific markers | Per clinical context | See Overview and Management tabs for condition-specific targets |
| I&Os | Strict if volume-sensitive | UOP โฅ 0.5 mL/kg/hr. Net fluid balance guides diuresis or resuscitation. |
| Telemetry | Continuous if indicated | Arrhythmia detection. Discontinue when no longer indicated (reduces alarm fatigue). |
| Clinical response | Each assessment | Symptom improvement, functional status, appetite, mental status -the exam matters more than labs |
| Drug | Dose | Route | Notes |
|---|---|---|---|
| Warfarin | Target INR 2-3 (arterial: 2.5-3.5) | PO | First-line for thrombotic APS. Lifelong after first event. DOACs are INFERIOR to warfarin in APS -TRAPS trial stopped early for excess arterial events with rivaroxaban TRAPS, 2018. ASTRO-APS confirmed: use warfarin, not DOACs. |
| Heparin (LMWH) | Enoxaparin 1 mg/kg BID | SQ | Acute thrombosis treatment, bridge to warfarin. Also used in obstetric APS. |
| ASA | 81 mg daily | PO | Primary prevention in asymptomatic aPL-positive patients (especially with SLE). Added to warfarin for arterial events. |
| Hydroxychloroquine | 200-400 mg daily | PO | Reduces thrombotic risk in SLE-associated APS. Antithrombotic + immunomodulatory properties. Consider in all APS patients. |
| Obstetric APS | |||
| ASA + LMWH | ASA 81 mg + enoxaparin 40 mg daily | PO/SQ | Standard regimen for obstetric APS (recurrent pregnancy loss, preeclampsia). Start ASA preconception, add LMWH at positive pregnancy test. |
| Catastrophic APS (CAPS) | |||
| Triple therapy | Anticoag + steroids + PLEX or IVIG | IV | CAPS = multi-organ failure in < 1 week. ~50% mortality. Anticoagulation + methylprednisolone 1g ร 3d + plasma exchange. Add rituximab or eculizumab if refractory. |