What is Antiphospholipid Syndrome?
Antiphospholipid Syndrome (APS) is an autoimmune thrombophilic disorder characterised by the persistent presence of antiphospholipid antibodies (aPL) — specifically Lupus Anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-β2-glycoprotein-I antibodies (anti-β2GPI) — in association with clinical events including arterial or venous thrombosis and/or pregnancy morbidity (recurrent miscarriages, stillbirths, or severe pre-eclampsia).
APS can occur in isolation (Primary APS) or in the context of another autoimmune disease, most commonly SLE (Secondary APS, present in 30-40% of lupus patients). The aPL antibodies activate platelets and endothelial cells, promote a prothrombotic state, and can cause catastrophic multi-organ thrombosis in its most severe form (Catastrophic APS or CAPS).
Deep vein thrombosis (DVT) — one of the most common thrombotic presentations of Antiphospholipid Syndrome.
Clinical Manifestations
APS is a truly multi-system disorder, affecting multiple vascular beds:
- Venous Thromboembolism: Deep Vein Thrombosis (DVT) and Pulmonary Embolism (PE) are the most common thrombotic events.
- Arterial Thrombosis: Stroke and Transient Ischaemic Attack (TIA) — particularly in young people (under 45 years) without conventional cardiovascular risk factors.
- Obstetric APS: Three or more consecutive early miscarriages (<10 weeks), one or more foetal deaths after 10 weeks, or premature birth due to severe pre-eclampsia or placental insufficiency.
- Livedo Reticularis: A net-like, purplish mottling of the skin — a vascular skin manifestation particularly associated with Sneddon's syndrome.
- Cardiac Manifestations: Libman-Sacks endocarditis (non-infective valve vegetations), cardiac valve disease.
- Thrombocytopenia: Low platelet count — paradoxically increases bleeding risk alongside the thrombotic tendency.
Livedo reticularis — a characteristic net-like skin discolouration frequently seen in APS patients.
🚨 Catastrophic APS (CAPS)
Catastrophic APS is a rare but life-threatening variant where widespread thrombosis develops simultaneously in multiple small vessels over less than a week, causing rapid multi-organ failure. Mortality is approximately 50%. Precipitants include infections, surgery, or medication withdrawal. It requires emergency treatment with anticoagulation, high-dose steroids, plasma exchange, and IVIG.
Dr. Prateek's Approach to APS
APS sits at the interface of Rheumatology, Haematology, and Obstetrics. Dr. Prateek Deo coordinates care across these specialties, implementing the appropriate anticoagulation strategy based on antibody profile, thrombotic history, and risk stratification.
Risk Stratification
We use the Global APS Score (GAPSS) and antibody profile (triple positivity indicates highest risk) to determine the intensity of anticoagulation and thromboprophylaxis needed.
Obstetric APS Management
Women with obstetric APS are managed with aspirin and low molecular weight heparin (LMWH) throughout pregnancy — a regimen that dramatically improves live birth rates.
Long-term Anticoagulation
Patients with thrombotic APS require indefinite anticoagulation with Warfarin (targeting INR 2.5-3.5) or DOACs under careful monitoring to prevent recurrent clots.
Associated SLE Screening
All APS patients are screened for SLE, as secondary APS requires treatment of the underlying autoimmune disease in addition to anticoagulation.