Professor Graham Hughes is a Consultant Rheumatologist and the founder and editor of the international journal ‘LUPUS,’ and Head of London Lupus Centre. In 1983 he described the clotting disorder now known as Hughes Syndrome for which he received the World Rheumatology (ILAR) Research Prize in 1993.

Author Quotes

I would just remind you that the gut is associated with APS as well, certainly gastric ischaemia or gut ischaemia – (intestinal) angina.

So the lessons are here – cerebral artery stenosis, renal artery stenosis, coeliac artery stenosis – these are all features that we see and this gives rise to more intensive looks at whether anticoagulation is the right treatment for these patients.

What’s the lesson? The lesson here is that the ENT doctors are probably seeing it, and we are being referred patients with Meniere’s and balance disorders: vertigo and ataxia as presentations of APS.

Summary

Continuing with the next video excerpt, Professor Hughes presents another three case studies from his clinic.

The first case study is of a 37-year old woman with coeliac artery stenosis and discusses several cases where focal narrowings seen on imaging are thought to be recanalized thrombosis. 

Moving on to the renal implications of APS, he discusses the focal lesions found in a girl who had hypertension, variable blood pressure, livido reticularis, and marked focal arterial kidney disease. The effect on the kidney by major vein thrombosis and microvascular thrombosis is presented. 

In case study 4, he presents the broadening clinical spectrum using data from a 61-year old female patient with APS and tinnitus, who was treated with anticoagulation.

Moving on to case study 5, Prof Hughes provides details of an elderly male patient with intermittent claudication, headaches, and migraine and discusses the genetics of APS.

Take-home Points

• Migraine and stroke are possibly major missing links with APS
• Genetic factors are likely important in APS
• The clinical spectrum of APS is broadening

Quiz

Learn more

1. Antiphospholipid Syndrome (APS) Case Files (Stroke and Thrombosis) By Prof Graham Hughes
2. Antiphospholipid Syndrome (APS) Case Files (Stillbirths and Epilepsy) By Prof Graham Hughes
3. Antiphospholipid Syndrome (APS): The Clinical Experience – Dr. Graham Hughes
4. Antiphospholipid Syndrome: 30 Years On – Dr. Graham Hughes
5. Central Nervous System (CNS) Involvement in Antiphospholipid (Hughes) Syndrome – Prof Graham Hughes
6. Clues to the Diagnosis of Antiphospholipid Syndrome – Prof Graham Hughes
7. Neuropsychiatric Lupus and The Curious Case of Molly – What Does This Case Teach Us? – Prof Hughes
8. Pathogenesis of Neuropsychiatric Systemic Lupus Erythematosus – Prof Graham Hughes
9. What is Lupus? – Prof Graham Hughes
10. Prof Graham Hughes Introduces You To The World of Lupus and Antiphospholipid Syndrome – Prof Graham Hughes
11. APS and CNS Lupus: Anticoagulation Treatment By Prof Graham Hughes
12. Neuropsychiatric Manifestations in APS By Prof Graham Hughes
13. Q & A session on Lupus, Hughes Syndrome and the Brain – Prof Hughes

 References

1. Sangle SR, Jan W, Lau IS, Bennett AN, Hughes GR, D’Cruz DP. Coeliac artery stenosis and antiphospholipid (Hughes) syndrome/antiphospholipid antibodies. Clin Exp Rheumatol. 2006 May-Jun;24(3):349.
2. Sangle SR, D’Cruz DP, Abbs IC, Khamashta MA, Hughes GR. Renal artery stenosis in hypertensive patients with antiphospholipid (Hughes) syndrome: outcome following anticoagulation.Rheumatology (Oxford). 2005 Mar;44(3):372-377. Erratum in: Rheumatology (Oxford). 2005 Apr;44(4):569.
3. Hughes GR.Antiphospholipid syndrome, migraine, and stroke.Lupus. 2010 Apr;19(5):555-556.