Clinical Review

Diagnosis and management of the antiphospholipid syndrome

BMJ 2010; 340 doi: http://dx.doi.org/10.1136/bmj.c2541 (Published 14 May 2010) Cite this as: BMJ 2010;340:c2541
  1. Danielle Cohen, research fellow1,
  2. Stefan P Berger, nephrologist2,
  3. Gerda M Steup-Beekman, rheumatologist, internist3,
  4. Kitty W M Bloemenkamp, maternal fetal medicine specialist4,
  5. Ingeborg M Bajema, pathologist1
  1. 1Department of Pathology, Leiden University Medical Centre, PO Box 9600, 2300 RC Leiden, Netherlands
  2. 2Department of Nephrology, Leiden University Medical Centre and Department of Internal Medicine, Haga Teaching Hospital, PO Box 40551, 2504 LN the Hague, Netherlands
  3. 3Department of Rheumatology, Leiden University Medical Centre and Bronovo Hospital, PO Box 96900, 2509 JH the Hague
  4. 4Department of Obstetrics, Leiden University Medical Centre
  1. Correspondence to: D Cohen d.cohen{at}lumc.nl

    Summary points

    • If untreated, antiphospholipid syndrome can lead to permanent disability, severe maternal or perinatal morbidity, or even death

    • Symptoms can occur in virtually all organ systems

    • Venous thrombosis and stroke are the most common thrombotic manifestations

    • In pregnancy the syndrome is associated with adverse maternal and fetal outcomes

    • The lupus anticoagulant test is the most useful because positivity correlates most strongly with clinical manifestations

    • Cardiac valvular disease is an important clinical manifestation and may contribute to the risk of stroke

    Antiphospholipid syndrome was first described 27 years ago in patients with systemic lupus erythematosus (SLE) and positive anticardiolipin antibodies, who presented with a clotting syndrome that affected arteries and veins.1 Female patients had a high risk of recurrent miscarriage and late fetal loss. The international classification criteria for this syndrome used today are based on those initial clinical observations.2

    The syndrome is under-recognised and underdiagnosed and can have devastating consequences if untreated, mainly because of uncontrolled thrombosis. Difficulties in diagnosis are compounded by a lack of standardisation of diagnostic tests. Early recognition is crucial, because treatment can reduce mortality and morbidity in relatively young people who often present with diseases such as stroke, myocardial infarction, and deep vein thrombosis.

    Because of its variable clinical presentation, patients with antiphospholipid syndrome present to a variety of medical practitioners. Here, we introduce this complicated and intriguing syndrome, and provide basic guiding principles for the recognition, diagnosis, and management of affected patients.

    Sources and selection criteria

    We searched the following databases for evidence from systematic reviews, clinical trials, and prospective cohort studies: PubMed (1949 to January 2010), Embase (1980 to January 2010), Web of Science (1945 to January 2010), Cochrane Library (1990 to January 2010), CINAHL (1982 to January 2010), and Academic Search Premier (1865 to January 2010). All relevant keyword variations were used. In general, the …

    Sign in

    Free trial

    Register for a free trial to thebmj.com to receive unlimited access to all content on thebmj.com for 14 days.
    Sign up for a free trial

    Subscribe