My apologies for the delay with the blog. This year’s annual ‘Ten Topics’ meeting here in London was huge! So stimulating: I’ll tell you more.

So far, at least here in London and Kent, July has been wonderful. Blue skies, little rain, warm weather. The tourist boats chugging up and down the Thames outside my clinic window at London Bridge Hospital are packed with visitors – London is awash with languages.

This month I received an honour of which I am so proud. My colleague in Portugal (Dr Jorge Martins) has opened a new clinic for lupus and related diseases, and has named it‘The Graham Hughes Clinic for Autoimmune Diseases’.

On Thursday and Friday, 2nd & 3rd July, we held the 29th annual meeting “Ten Topics in Rheumatology”. This yearly meeting, now also held regularly by colleagues in Barcelona, Nice, Singapore, Beirut and Buenos Aires, is designed to provide a comprehensive ‘update’ in rheumatology and APS, both to consultant and trainee doctors. We had two hundred and fifty attendees, coming from as far afield as Australia, Malaysia, Iraq, as well as a group of 12 doctors from India – 11 countries, in all – and the feedback has been fantastic.

The meeting covered advances in all aspects of rheumatology, with speakers from the UK, Spain, Israel, Holland and Belgium. My friend, Professor Yehuda Shoenfeld from Israel spoke about the use of intravenous immunoglobulin (I.V.I.G.) and our main guest lecturer, Professor Paul-Peter Tak (Amsterdam) talked about the links between the brain and the joints.

The session on Hughes Syndrome (sponsored by INOVA, a US firm innovating lab tests for Hughes Syndrome), included an update by Professor Khamashta on the new oral anticoagulants (‘early days’) and by Dr Ricard Cervera (Barcelona) on insights into the life-threatening ‘catastrophic APS’ – a condition involving widespread clotting.

My own contribution, as organiser, was small – a presentation ‘highlighting’ the importance of brain/neurological presentations of Hughes Syndrome in 3 patients, and the lessons to be learnt. Here are the 3 patients:

Patient(s) of the Month

Case 1: Mrs M D, aged 30, suffered from ‘glandular fever’ at the age of 15. Following this, she suffered from severe sleep disturbance – so bad that she went on to develop ‘narcolepsy’ (falling asleep at odd times) and even ‘cataplexy’ (suddenly falling down asleep). When referred to my clinic, she was found to have livedo (corned beef skin) and very dry eyes, suggestive of Sjogren’s Syndrome. She had high titres of anticardiolipin antibodies.

She was started on low dose aspirin, and later Plavix. The improvement was both dramatic and immediate.

Case 2: Ms D L, aged 15, suffered from an attack of ‘glandular fever’, following which she developed auditory hallucinations and chorea (movement disorders) – so severe that at times her whole body was writhing. She was found to be positive for one of the 3 ‘aPL’ tests – Beta 2 GP1. A trial of heparin produced partial improvement – but full ‘cure’ proved difficult. Finally, a course of treatment with intravenous immunoglobulin (I.V.I.G.) was undertaken. Complete resolution of the St Vitus Dance!

Case 3: Mrs M J, aged 41, had been diagnosed with mild lupus ten years previously. Her anti-DNA test was borderline but her aCL level was sky high.

Her main clinical problem, however, was not so much the lupus but more her epilepsy – requiring the care of a specialised epilepsy unit and at least 4 anti-epileptic drugs.

Two years ago, she developed a leg clot (DVT) and was anti-coagulated with warfarin. Dramatic improvement in epilepsy – down to 1 anti-epileptic drug for the past 2 years! (we have, of course continued the warfarin).

What are these 3 patients teaching us?

At least 5 possible lessons – glandular fever, sleep pathology, movement disorder (chorea), epilepsy, and the sometimes unexpected success of treatment.

Glandular fever (E.B. virus), is an odd condition, common in teenage years. There is no doubt that it can trigger autoimmune diseases such as Sjogren’s syndrome and lupus.

Secondly, Hughes Syndrome and the brain. It has always been my teaching that Hughes Syndrome can be a predominantly ‘neurological’ condition, with stroke, migraine and memory loss being common, but with other features such as epilepsy, sleep disturbance and movement disorders also well recognised.

In summary, I suppose the 2 overriding take-away messages are:

1. Hughes Syndrome is not just miscarriage and thrombosis

2. (For doctors) – If you suspect that Hughes Syndrome (APS) is the root cause of the patient’s symptoms, don’t be afraid to treat!

Professor Graham R V Hughes MD FRCP