Twenty years ago, in the international journal LUPUS (http://www.sagepublications.com), I introduced a regular series called “Lupus around the world”, publishing reports on lupus prevalence and clinical features in different countries. The series, immensely popular, has become a regular feature in LUPUS, providing important data from every corner of the world. We are now publishing a collection of these articles which, I hope, will provide useful data for those wishing to study this important, worldwide disease.
Listen to the patient!
The clinical diagnosis carries far more weight than the blood tests.
So how common is Hughes Syndrome? Judging by the figures published over the last three decades, VERY common. Obviously, figures will vary widely – take migraine for example. In my view, this is one of the commonest features of Hughes syndrome – yet the true prevalence of positive antiphospholipid antibodies (aPL) – and of possible Hughes syndrome - in those patients attending migraine clinics is unknown.
I often tell my students that Hughes Syndrome is first and foremost a neurological disease. The two organs seemingly most sensitive to ‘sticky blood’ are the placenta in pregnancy and the brain. Almost any ‘neurological’ feature can be seen in Hughes Syndrome, ranging from the common – migraine, memory loss, ‘funny turns’, TIAs and balance problems to the less common – ‘atypical’ MS, Stroke, visual disturbance, seizures and finally to the relatively rare – tics, movement disorders and even Tourette’s Syndrome.
I have just received a copy of our latest book, ‘Sjögren’s Syndrome in Clinical Practice’. Produced with colleagues Dr Shirish Sangle and Dr Simon Bowman, it is published by Springer (ISBN 978-3-319-06058-3).
I hope it will prove helpful both to patients as well as to doctors. One of the aims of the book was to highlight the overlap of Sjögren’s syndrome with thyroid problems, gluten sensitivity and, something largely missing in previous Sjögren’s books, the overlap with Hughes syndrome (headaches, memory loss, TIAs, bone fracture, etc).
Many of you, having read these blogs over the past few years, will have recognised that migraine pops up time and again as a feature of Hughes Syndrome. Often severe and ‘hemiplegic’, the migraine tendency in Hughes Syndrome can go back to childhood or the teens and commonly, and very dramatically, can be cured in Hughes Syndrome patients with blood thinning treatment. And yet, sadly, the link goes unrecognised. There is an even more important message: it has long been recognised that patients with migraine have an increased risk of TIA and stroke – all three being known features of Hughes Syndrome.
“The serious facts about funny turns”.
So ran the headline in one of this week’s free newspapers – the Metro. In many ways it highlights some of the problems faced by Hughes Syndrome patients.
One would have assumed that simple anti-phospholipid (aPL) testing would now be routine in all pregnancies – or, if not, certainly after a miscarriage. Unfortunately not - the reason being that there are, of course, many known causes of miscarriage. It is therefore widely accepted that routine aPL testing is only required after three miscarriages! I can see the (economic) arguments for this, but I am fighting to encourage more widespread earlier testing.
In this month’s blog I propose to deal (or to start to deal) with ‘treatment’ – still a major issue for all of us – patients and doctors, living with Hughes Syndrome.
In March, I have been asked to give a talk at the annual world conference on autoimmune diseases, held this year in France. The title they have given me is interesting – ‘APS/Hughes Syndrome – fact and fiction’. Which leads me to the important message in this month’s blog – NEGATIVE TESTS DO NOT RULE OUT HUGHES SYNDROME.
Sadly, I am seeing a number of Hughes Syndrome patients who have not only been passed from pillar to post but, in the case of some patients, discharged from clinic – an attitude which is unforgivable.