Antiphospholipid syndrome

Last reviewed: 26 Apr 2022
Last updated: 07 May 2021

Summary

Definition

History and exam

Key diagnostic factors

  • history or current diagnosis of vascular thrombosis
  • history of pregnancy loss
  • history of pregnancy-associated morbidity
More key diagnostic factors

Other diagnostic factors

  • history of systemic lupus erythematosus
  • features of thrombocytopenia
  • arthralgia/arthritis
  • livedo reticularis
  • history of other rheumatological disorders or connective tissue disorders
  • cardiac murmur
  • oedema
  • seizure, headache, memory loss, signs of transverse myelopathy
  • limb discomfort, swelling, skin discoloration, and ulcers
Other diagnostic factors

Risk factors

  • history of systemic lupus erythematosus
  • history of other autoimmune rheumatological disorders
  • history of other autoimmune diseases
  • history of autoimmune haematological disorders
More risk factors

Diagnostic investigations

1st investigations to order

  • lupus anticoagulant
  • anticardiolipin antibodies
  • anti-beta2-glycoprotein I antibodies
  • antinuclear antibody, double-stranded DNA, and extractable nuclear antigen antibodies
  • FBC
  • creatinine and urea
More 1st investigations to order

Investigations 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
More investigations to consider

Treatment algorithm

ACUTE

non-pregnant with acute thrombosis

pregnant

catastrophic APS (pregnant or non-pregnant)

ONGOING

incidental antiphospholipid antibodies

Contributors

Authors

Karen A. Breen, MB, MRCPI

Consultant Haematologist

Guys and St. Thomas' Foundation Trust

London

UK

Disclosures

KAB has performed consultancy work for Bayer/Pfizer.

Beverley J. Hunt, MD, FRCP, FRCPath

Professor of Thrombosis and Haemostasis

King's College

Guys and St. Thomas' Foundation Trust

London

UK

Disclosures

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's syndrome
    • Inherited thrombophilia
    More Differentials
  • Guidelines

    • 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
    • EULAR recommendations for the management of antiphospholipid syndrome in adults
    More Guidelines
  • Patient leaflets

    Deep vein thrombosis

    Miscarriage

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

Use of this content is subject to our disclaimer