Clues to the Diagnosis of Antiphospholipid Syndrome – Prof Graham Hughes

Posted on May 6, 2017

Antiphospholipid syndrome (APS), which is also known as sticky blood or Hughes syndrome, was first fully described 30 years ago as a syndrome that involves arterial and venous thrombosis with prominent cerebral involvement. The main reported features of this syndrome include migraines, chorea, epilepsy, and cerebrovascular accidents.  Initially, the syndrome was considered to be a distinct entity, called anticardiolipin syndrome in 1985, and it was later renamed antiphospholipid antibody syndrome. APS is an autoimmune disease in which autoantibody production results in a hypercoagulable state that causes recurrent arterial and venous thrombosis and/or recurrent foetal loss.

Psychiatric illness may be the initial manifestation of the syndrome, requiring psychiatrists to be vigilant for the occurrence of the syndrome due to its potential preventive and therapeutic implications.

Prof Graham Hughes who discovered APS and has the syndrome named after him takes us through some pointers to diagnose this great mimic.

Doctor, do you have any patients with migraine and headache, DVT, recurrent miscarriage, memory loss, TIA and young strokes? – These are all features of Hughes Syndrome.

Clinical Pointers to APS:

  • Teenage migraine – “so important and so common.”
  • Tendency to thrombosis – “interestingly in arteries as well as veins.”
  • TIAs, strokes, memory loss
  • Recurrent miscarriage, including LATE pregnancy loss
  • Cold ‘blotchy’ circulation
  • Giddiness and ataxia
  • Visual disturbance
  • Atypical multiple sclerosis
  • Venous leg ulcers
  • Occasional low platelets
  • Occasional heart murmurs

Laboratory Criteria for Diagnosis: (Miyakis et al.,)
1. The presence of lupus anticoagulant (LA) in the plasma on two or more occasions at least 12 weeks apart.
2. The presence of the anticardiolipin (aCL) antibody of immunoglobulin (Ig)G and/or IgM isotype in the serum or plasma in medium or high
titre (i.e. 40 GPL units or MPL units, or the 99th percentile) on two or more occasions at least 12 weeks apart.
3. The presence of the anti-b2-glycoprotein I antibody of IgG and/or IgM isotype in the serum or plasma (in titres!the 99th percentile) on two or more occasions at least 12 weeks apart.

A positive test of Anti-Cardiolipin predicts 80% of cases, Lupus Anticoagulant predicts 30-40% of cases… the tests should be done together if you’re ticking the boxes and looking for this syndrome.

Want to learn more? Prof Graham Hughes introduces you to the world of Lupus and Antiphospholipid Syndrome.

Download the literature review on Antiphospholipid Antibodies in Psychiatry.