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Antiphospholipid Syndrome (APS)

Acquired thrombophilia with vascular thrombosis and pregnancy morbidity.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Antiphospholipid Syndrome (APS)?

APS is caused by pathogenic autoantibodies directed against phospholipid-binding plasma proteins, particularly β2-glycoprotein I and prothrombin.

These antibodies promote a procoagulant state by activating platelets and endothelial cells, inhibiting natural anticoagulants and triggering complement activation.

APS occurs as a primary disorder or secondary to systemic lupus erythematosus and other connective tissue diseases; a subset develops catastrophic APS, a multi-organ thrombotic storm with mortality near 50%.

Symptoms

Venous thrombosis: deep vein thrombosis and pulmonary embolism (most common manifestation)
Arterial events: stroke, transient ischaemic attack, myocardial infarction, retinal artery occlusion, limb ischaemia
Pregnancy morbidity: three or more consecutive early miscarriages before 10 weeks, fetal death after 10 weeks, premature birth before 34 weeks due to severe preeclampsia or placental insufficiency
Thrombocytopenia and autoimmune haemolytic anaemia
Livedo reticularis on skin examination
Valvular heart disease (Libman-Sacks endocarditis)
Cognitive dysfunction, chorea and migraine
Catastrophic APS: multi-organ failure with widespread small-vessel thrombosis

Risk Factors

Female sex (3-5 times more common than males)
Systemic lupus erythematosus and other connective tissue diseases
Family history of autoimmune disease
Prior unprovoked thromboembolism or recurrent miscarriage
Triple antibody positivity (lupus anticoagulant + anticardiolipin + anti-β2GPI)
High antibody titres and IgG isotype
Co-existing cardiovascular risk factors (hypertension, smoking, oestrogen therapy)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Any unprovoked venous or arterial thrombosis in a young patient warrants APS workup
  • Two or more spontaneous pregnancy losses or one fetal death after 10 weeks needs antibody testing
  • New neurological deficit, chest pain or limb ischaemia in a known APS patient is a medical emergency
  • Pregnancy planning in known APS patients requires high-risk obstetric referral
  • Suspicion of catastrophic APS (multi-organ failure with thrombosis) demands immediate intensive care admission

Treatment Methods

01
Long-term anticoagulation with warfarin (target INR 2.0-3.0 for venous events; 3.0-4.0 for arterial or recurrent events)
02
Direct oral anticoagulants are contraindicated in triple-positive patients due to higher recurrence rates
03
Obstetric APS: low-dose aspirin plus prophylactic low-molecular-weight heparin throughout pregnancy and 6 weeks postpartum
04
Hydroxychloroquine, statins and vitamin D supplementation for additional benefit
05
Catastrophic APS: combined anticoagulation, high-dose corticosteroids, plasmapheresis, intravenous immunoglobulin
06
Refractory cases: rituximab or eculizumab
07
Lifestyle measures: avoid oestrogen-containing contraceptives, control cardiovascular risk factors, smoking cessation

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.