When viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.

Antiphospholipid syndrome

Last reviewed: 12 Nov 2024
Last updated: 16 Aug 2024

Summary

Definition

History and exam

Key diagnostic factors

  • history or current diagnosis of vascular thrombosis
  • history of pregnancy loss
  • history of pregnancy-associated morbidity
Full details

Other diagnostic factors

  • history of systemic lupus erythematosus
  • features of thrombocytopenia
  • arthralgia/arthritis
  • livedo reticularis
  • history of other rheumatologic disorders or connective tissue disorders
  • cardiac murmur
  • edema
  • seizure, headache, memory loss, signs of transverse myelopathy
  • limb discomfort, swelling, skin discoloration, and ulcers
Full details

Risk factors

  • history of systemic lupus erythematosus
  • history of other autoimmune rheumatologic disorders
  • history of other autoimmune diseases
  • history of autoimmune hematologic disorders
Full details

Diagnostic tests

1st tests to order

  • lupus anticoagulant
  • anticardiolipin antibodies
  • anti-beta2-glycoprotein I antibodies
  • antinuclear antibody, double-stranded DNA, and extractable nuclear antigen antibodies
  • CBC
  • creatinine and BUN
Full details

Tests to consider

  • venous Doppler ultrasound
  • venography or MRI
  • MRI of thrombosis
  • CT angiogram of the chest
  • ventilation-perfusion (V/Q) scan
  • cranial MRI
  • echocardiography
  • inherited thrombophilia test
Full details

Treatment algorithm

ACUTE

nonpregnant with acute thrombosis

pregnant

catastrophic APS (pregnant or nonpregnant)

ONGOING

incidental antiphospholipid antibodies

Contributors

Authors

Damon Houghton, MD, MS

Associate Professor of Medicine

Mayo Clinic

Gonda Vascular Center

Department of Internal Medicine, Division of Hematology & Department of Cardiovascular Diseases, Division of Vascular Medicine

Rochester

MN

Disclosures

DH has received clinical research support from Veralox and Bayer.

Ana I. Casanegra, MD, MS

Associate Professor of Medicine

Mayo Clinic

Gonda Vascular Center

Department of Cardiovascular Diseases, Division of Vascular Medicine

Rochester

MN

Disclosures

AC declares that she has no competing interests.

Alí Duarte-García, MD, MSc

Assistant Professor of Medicine

Mayo Clinic

Department of Internal Medicine, Division of Rheumatology

Rochester

MN

Disclosures

AD declares that he has no competing interests.

Acknowledgements

Dr Damon Houghton, Dr Ana I. Casanegra and Dr Alí Duarte-García would like to gratefully acknowledge Dr Karen A. Breen and Dr Beverley J. Hunt, the previous contributors to this topic. KAB has performed consultancy work for Bayer/Pfizer. BJH is Medical Director of Thrombosis UK and chair of the steering committee for World Thrombosis Day. BJH is an author of a reference cited in this topic.

Peer reviewers

Kuntal Chakravarty, FRCP (London), FRCP (Glasgow), FRCP (Ireland), FACP (USA), FACR (USA)

Consultant Rheumatologist

BHRT University Hospital

Queen’s Hospital

Romford

UK

Disclosures

KC declares that he has no competing interests.

Alan Lichtin, MD

Staff Hematologist

Cleveland Clinic Taussig Cancer Center

Cleveland

OH

Disclosures

AL declares that he has no competing interests.

  • Antiphospholipid syndrome images
  • Differentials

    • Sneddon syndrome
    • Inherited thrombophilia
    More Differentials
  • Guidelines

    • ACR/EULAR antiphospholipid syndrome classification criteria
    • Addendum to British Society for Haematology Guidelines on Investigation and Management of Antiphospholipid syndrome, 2012 (Br. J. Haematol. 2012; 157: 47–58): use of direct acting oral anticoagulants
    More Guidelines
  • Patient information

    Deep vein thrombosis

    Miscarriage

    More Patient information
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer