I am writing this blog on the last day of the year – an eventful year both in politics and, for me, in medicine. For me the highlight of the year was the opening of the Graham Hughes Autoimmune Diseases clinic in Madeira. This honour was especially cherished as it came from another country, Madeira, Portugal and arrived completely out of the blue.
Good and bad! November saw the publication of the 2nd edition of my book “Hughes Syndrome. A patient’s Guide”. Wonderful improvement, stories of two of my patients – one from the Scottish Isles, and one from Romania.
The first patient from the Scottish Isles presented with severe neurological problems. Treated by Dr Hoda El-Mahrouki with heparin and then warfarin (INR 3.5-4) and now completely well. The Romanian patient is this month’s patient of the month.
As far as Hughes Syndrome is concerned, there are still major challenges – the best anticoagulant, what to think when the tests are negative yet the clinical features strongly point to the diagnosis, and the screening for ‘sticky blood’ in pregnancy – to name but three.
Another ‘challenge’ concerns the causes (and treatment) of ‘funny turns’ in Hughes Syndrome – the topic of this month’s ‘patient of the month’.
The majority opinion regarding the new anticoagulants was that the jury is still out. Hughes Syndrome patients often seem to require more aggressive warfarin treatment (e.g., INR between 3.5 and 4) than others attending anticoagulant clinics. Although increasing numbers of patients are on the new drugs, more data is needed. The good news is that a number of international trials are in progress.
Many years ago, an eye specialist named Hendrick Sjogren reported on a small group of patients who had a ‘triad’ of dry eyes, dry mouth and aches and pains.
Sjogren’s Syndrome most commonly affects an older age group of women than lupus. As well as the fatigue, there is often a history of allergies (including in some patients, gluten sensitivity). Blood tests often show a positive ANA. Critically the high level of anti-DNA antibodies, so characteristic of lupus, is absent.
Many colleagues think of Hughes Syndrome as ‘blood clots and miscarriage’. However, it is clear that the symptoms can be varied and widespread. “Sticky blood” can, quite obviously, affect any organ, including the heart (angina), the gut (abdominal pain after eating), and, in the case of Mrs F.D., the lungs, for example, from multiple small pulmonary emboli.
Could “sticky blood” affect the oxygen supply to the bones?
I took the opportunity to run an ‘anonymous’ survey of the patients with 4 questions: – How long did it take from the beginning of symptoms until diagnosis (rough estimate)? How many doctors/specialists did you see before diagnosis and treatment? What other diagnoses were you given? If you had had any pregnancies, how many pregnancy losses did you suffer?
Of course, such a straw poll has many defects, but the responses came up with some interesting findings.
A Jigsaw Puzzle
The Autoimmune Conference highlighted what we all suspect – that there is quite an overlap between many of these diseases. In previous blogs, I have often talked about “the big three” – Hughes syndrome, thyroid problems and Sjogren’s syndrome. This month my “Patient of the Month” illustrates how complex the links can be.
“Twins” - what can they teach us?
Out there in migraine clinics, in cardiology clinics, in miscarriage clinics, in multiple sclerosis clinics, in balance clinics, and even in (younger) memory loss clinics, there may be some patients – perhaps only a few – who do have Hughes Syndrome but with diagnosis and treatment taken no further because “the tests are negative.”
Late last year, I received an invitation from Dr Simon Helfgott (Boston), the editor of Rheumatology News, to write a major article on Hughes Syndrome. Now published, the article is clinically based, and, I hope it highlights the fact that not all of Hughes Syndrome is ‘thrombosis and miscarriage’ but includes features as varied as bone fracture, sleep disturbance and stillbirth (a topic I will cover in this month’s blog). The article is published in “The Rheumatologist” (Vol 10, No 2, Feb 2016) (http://www.therheumatologist.org) and has been well received – including the following email from Professor Gerry Weissmann, Dean Emeritus of New York University School of Medicine – one of my medical ‘heroes’ – “What a masterful article by a giant in our field…to be treasured.”