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Antiphospholipid Syndrome

Also Known As: Antiphospholipid Antibody Syndrome, Hughes Syndrome, APLS, Lupus Anticoagulant Syndrome
System: Heart and Vascular System, Immune System, Reproductive System, and Nervous System
Autoantibodies:

  • Lupus anticoagulant (LA)

  • Anti-cardiolipin antibodies (IgG, IgM)

  • Anti-beta-2 glycoprotein I antibodies (IgG, IgM)

Primary Organ or Body Part Affected:

  • Blood vessels (arteries and veins)

  • Placenta (during pregnancy)

Acceptance as Autoimmune: Confirmed
Antiphospholipid Syndrome

What is Antiphospholipid Syndrome?

Antiphospholipid Syndrome (APS) is an autoimmune disorder in which the body produces antibodies that mistakenly attack phospholipids—molecules that help blood flow smoothly. These antibodies increase the risk of abnormal blood clots in arteries and veins, leading to serious complications such as deep vein thrombosis (DVT), stroke, heart attack, and pregnancy loss. APS can occur on its own (primary APS) or in association with another autoimmune condition, most often systemic lupus erythematosus (SLE).

What are the signs and symptoms of Antiphospholipid Syndrome?

The most common symptoms relate to abnormal clotting and pregnancy complications:

Blood clot-related symptoms:

  • Deep vein thrombosis (pain, swelling, redness in the leg)

  • Pulmonary embolism (sudden chest pain, shortness of breath)

  • Stroke or transient ischemic attacks (TIA)

  • Heart attack

  • Retinal vein thrombosis (blurred vision or vision loss)

Pregnancy-related symptoms:

  • Recurrent miscarriages (usually in the 2nd or 3rd trimester)

  • Stillbirth

  • Preeclampsia or eclampsia

  • Intrauterine growth restriction (IUGR)

Other possible signs:

  • Livedo reticularis (mottled purplish skin rash)

  • Chronic headaches or migraines

  • Low platelet count (thrombocytopenia)

  • Cognitive or memory problems

  • Seizures (in rare cases)

What are the causes of Antiphospholipid Syndrome?

APS is caused by the presence of autoantibodies that mistakenly target phospholipid-binding proteins, especially beta-2 glycoprotein I and prothrombin, leading to abnormal clot formation. Triggers may include:

  • Genetic susceptibility

  • Infections (e.g., viral or bacterial)

  • Medications (e.g., hydralazine, phenytoin)

  • Other autoimmune diseases, particularly lupus

Diagnosis

Diagnosis requires both clinical criteria (evidence of blood clots or pregnancy complications) and laboratory criteria (positive antibody tests).

Required lab tests (must be positive on 2 occasions at least 12 weeks apart):

  • Lupus anticoagulant (LA)

  • Anti-cardiolipin antibodies (aCL)

  • Anti-beta-2 glycoprotein I antibodies

Additional tests may include:

  • Coagulation panels (may show prolonged aPTT despite increased clotting risk)

  • Imaging (e.g., Doppler ultrasound, CT angiogram) to detect clots

  • Pregnancy monitoring if applicable

Treatment

There is no cure, but treatment focuses on preventing blood clots and managing risks:

Standard treatment:

  • Lifelong anticoagulation with warfarin (target INR 2.0–3.0, or higher in high-risk cases)

  • Low-dose aspirin may be added

  • Heparin and aspirin during pregnancy (warfarin is avoided during pregnancy due to fetal risk)

Other treatments (in select cases):

  • Direct oral anticoagulants (DOACs) – generally not recommended for APS with high-risk antibodies

  • Hydroxychloroquine (in lupus-associated APS)

  • Statins or immunosuppressants in severe or refractory cases

Prognosis

  • With proper anticoagulation, many patients live full, healthy lives

  • Risk of recurrence is high without treatment (up to 50%)

  • In pregnant women, appropriate treatment significantly improves outcomes

  • Catastrophic APS (CAPS), a rare and severe form, carries a high mortality rate (30–50%) without immediate intensive care

Prevalence

  • 1–5% of the general population carry antiphospholipid antibodies

  • Symptomatic APS affects approximately 40–50 per 100,000 people

  • Women are affected 3–5 times more often than men

  • Present in 15–20% of all cases of deep vein thrombosis or stroke in young people

  • Up to 20–30% of lupus patients have APS

Citations

  • Cervera R, et al. (2009). Antiphospholipid syndrome. The Lancet, 373(9671), 1498–1509.

  • Miyakis S, et al. (2006). International consensus statement on an update of the classification criteria for APS. J Thromb Haemost.

  • Erkan D, et al. (2020). Management of thrombotic APS: current and emerging treatments. Rheum Dis Clin North Am.

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