Sticky Blood-Hughes Syndrome Support
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Prof Hughes' June 2011 blog

Although he has retired from the NHS, Professor Graham Hughes still keeps very active and is an avid blogger :) Below is his blog from June 2011:

- June 2011

A very late blog. I had privately planned to stop this month, but Kate flattered me by asking me to continue. So …

One of the main reasons for the delay is that there are major changes going on in the Louise Coote Lupus Unit at St Thomas’. It seems that those in power have decided to re-link the unit with rheumatology.

Although most of my colleagues there have, so far, received little or no information, things are already changing. The Louise Coote Lupus Unit will hold rheumatology clinics, and our lupus and Hughes Syndrome patients may be shunted to general rheumatology clinic in Guy’s Hospital, or to haematology on the fourth floor at St Thomas’. Some of this has already happened. This week the charity offices of the Hughes Syndrome Foundation and the St Thomas Lupus Trust moved to an empty ward in Guy’s Hospital – Kate and Angie have kept their old phone numbers but have little else at present.

Although I am long retired from St Thomas’, it saddens me to see the world famous unit being divided up – especially at a time when six major clinics across Europe are setting up units based on our model – a unit bringing together specialists and staff expertise in dealing with these complex disorders. And patients not having to travel from pillar to post …

Two interesting meetings this month. The first was our annual Ten Topics meeting at St Thomas’ Hospital. A very special meeting – the 25th anniversary. As always, the two day meeting (held this year on 30th June and 1st July) was fully booked, with doctors registering from all over Britain and the continent, and attendees from as far afield as Lebanon, Israel, South Africa and one brave doctor making it from Libya.

This meeting covers all the major advances in rheumatology, with especial emphasis on lupus and Hughes Syndrome. The 25th meeting, including a number of speakers making ‘comebacks’ from previous meetings was, in my view, the best ever.

The second meeting was rather different – a guest lecture in China (Changsha) – a whirlwind visit – travel out on Thursday/Friday, lecture on Saturday, home Sunday! My naïve theory is that you don’t get jet lag if you make the visit short!

The meeting was great, with over 1000 attendees – very keen interest in Hughes Syndrome. As with lupus, there appear to be very large numbers of people in China already diagnosed with Hughes Syndrome. And as with other aspects of medicine, there is a dramatic increase in the research effort into these two diseases, as evidenced by the exponential growth of papers submitted to the international journal LUPUS.

Case of the Month

A 30 year old man was referred by an Ear/Nose/Throat (ENT) colleague with a history of acute hearing loss in one ear. Interestingly, blood tests had turned up two positive tests for anticardiolipin antibodies (aCL). Could there be a connection?

The man’s previous health had been good, with nothing to suggest thrombosis or underlying disease. The family history threw up no clues. He had, however, suffered from severe migraine in his late teens and early twenties – often as frequently as one every third or fourth day.

Our own investigations proved intriguing. He had negative aCL and lupus anticoagulant (LA) tests on this occasion, but had a strongly positive anti-beta2-glycoprotein1 test.

We have started low dose aspirin, but may have to change to clopidogrel (Plavix) if there is any negative further effect on his hearing.

What is this patient teaching us?

ENT problems, especially balance problems, are becoming well recognised in Hughes Syndrome. Whether this patient’s hearing loss is a direct result of ‘sticky blood’ is of course uncertain, though his past history of severe migraine does suggest a clinical link with antiphospholipid antibodies (aPL).

For me, there is another lesson. Our testing for aPL is still imperfect – there are laboratory differences, and there are an increasing number of patients seen who have all the hallmarks of Hughes Syndrome, but with negative tests.

The development of newer tests is proceeding apace. One test now increasingly added to the two routine tests (aCL and LA) is the prosaically named anti-beta2-glycoprotein1 antibody test – usually shortened to ‘Beta 2’. While our experience so far has not turned up many ‘aPL-negative’ patients where the Beta 2 is the only positive clue, it certainly opens ones eyes. Watch this space.

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