Education, Awareness, Support

June 2018

by Professor Graham R V Hughes MD FRCP

What a month!  After such a gloomy and wet spring, all is forgiven.  June has been stunning.  Blue skies ever since the Royal Wedding, with temperatures in the 80’s.  The countryside glorious and London looking its very best.

On the home front, our new educational charity is now finally getting into full shape.

As part of our ‘introductory’ events, we are holding a 'Patients' Meeting on Friday, 21st September 2018 in the London Hilton Tower Bridge (opposite London Bridge Station), and topics will include Lupus and Hughes Syndrome.  For more details go to: http://bit.ly/2u02LYb

The meeting is being run in collaboration with Lupus UK and is being sponsored by London Bridge Hospital, and the afternoon will consist of short talks by both patients and doctors, followed by a ‘Question and Answer’ session.

On the ‘lectures’ side, I took part in 2 very interesting and fruitful meetings this month.  One in Madeira and 2 days later, one in Nashville, Tennessee. 

The meeting in Madeira was a ‘home from home’ occasion – though limited to 24 hours.  The host, Dr Jorge Martins, had previously named their Lupus clinic “The Graham Hughes Autoimmune Diseases Clinic”. 

This particular meeting, welcoming both doctors and patients, was aimed at introducing Madeira to the family of ‘Ten Topics’ hosts (now including Barcelona, Rome, Nice, Beirut, Singapore, Hong Kong and Buenos Airies, and most recently, India).

Speakers included my good friends Carlos Vasconcelos (Porto), as well as Carlos Diaz (Lisbon), David D’Cruz, Munther Khamashta, Ricard Cervera (Barcelona) and David Isenberg.

For me, it was a dash home – overnight, then a trip to Nashville the next day.  Why Nashville?  Because here was a 600 strong gathering of patients and doctors dealing with so-called ‘autonomic nerve’ diseases – or, as the congress was called “Dysautonomia” (good for scrabble?).

I must say, it was an enormous learning curve.  The autonomic nervous system is a rather mysterious network of nerves, which supply pretty well all the organs of the body – the intestines, the heart, the skin, the blood vessels – and so on.

As you can imagine, when the nerve network goes wrong, the effects can be devastating – and complex.  Features include bowel disturbance (in some cases requiring ‘artificial feeding’), blood pressure and pulse fluctuations (e.g., fainting on stand up), sweating (or the absence of sweating) and so on.  And links to Hughes Syndrome?  This month’s Patient of the Month may give some clues. 1

 

Patient of the Month

Mrs M.E. aged 45, had a long and complicated history.  She brought with her a drawing illustration of her symptoms.  These included headaches, fluctuating blood pressure (and a tendency to faint on standing), dry mouth, joint pains, blurred vision, stomach pains, numbness in the hands and feet, and balance problems.

She had been diagnosed with POTS (‘ Postural Orthostatic Tachycardia Syndrome’) and had been under the care of the cardiologists.  Treatment had been complex, with control of fluctuating blood pressure, fluid balance and worsening bowel function requiring close monitoring.

The cardiologist had suspected Hughes Syndrome (the patient had had a previous DVT and pregnancy losses).  Blood tests had revealed positive anti-nuclear antibodies and a strongly positive test for anticardiolipin antibodies.

She had been started on low dose aspirin, but with little benefit.  The diagnosis of autoimmune disease (Hughes Syndrome, probable Sjogren’s and autonomic nerve involvement) was made.  In view of the severity of the illness, and the lack of response to aspirin, we decided on our old standby – a 2 to 3 week trial of daily low dose heparin.

The result was striking – I quote the email sent by the patient:

“……within 48 hours of starting heparin….

       headache – gone

       tinnitus – gone

       stomach pains – gone

       numbness in hands – gone

       heart palpitations – gone

       balance – better

       visual disturbance – better

       brain fog – 50% improved

(None of these had responded to POTS medicines)

 

What is this patient teaching us?

This was the dramatic start of what has become an exciting whole new chapter in the Hughes Syndrome story.  Together with Jill Schofield, a Denver physician, we published a study of 14 similar cases.  The link with POTS has proved to be another strand in the association with autoimmune disease, such as Sjogren’s.

The Nashville meeting brought together researchers from all over the USA and Canada.  My role was to explain Hughes Syndrome and antiphospholipid antibodies.

My reward was to learn so much about the symptoms, signs and treatment of autonomic nerve disorders, both from the visiting colleagues as well as from the patients at the meeting.

So the lesson from today’s ‘Patient of the Month’; Hughes Syndrome can, in some cases, be linked to other autoimmune diseases such as Lupus, low Thyroid, Sjogren’s.  And now with ‘Dysautonomia.’  Never simple!

 

1 Taken from the article “Hughes Syndrome/APS: 30 years on, what have we learnt.  Opening talk at the 14th International Congress on antiphospholipid antibodies in Rio de Janeiro.  October 2013 (LUPUS 2014, 23, 400-405)

To read the talk in full, please go to the Lupus Journal page of the GHIC website:

https://ghic.world/lupus-journal/index.php

Professor Graham R V Hughes MD FRCP

Professor Graham R V Hughes MD FRCP

Head of The London Lupus Centre
London Bridge Hospital