Likewise in Hughes Syndrome, classification criteria have been produced by a committee of doctors – useful for uniformity in international collaborative studies, but definitely not for diagnosis. Clear?
The second lesson concerns duration of treatment. As in other patients, attempts to stop anticoagulation treatment met with a return of symptoms – in Joan’s case, worrying headaches.
Firstly, that there may be more than one diagnosis. Secondly, the triad of low thyroid, Sjogrens Syndrome and Hughes Syndrome commonly occur together. As these are classified as autoimmune diseases it is therefore not surprising that there should be overlap. (Anecdotally, it is also quite common for one or more of these conditions to be found in family members of our patients).
I make no apologies for coming back to the topic of migraine. It needs to be shouted from the roof tops that 1) migraine is a common feature of Hughes Syndrome and 2) treatment of Hughes Syndrome (with aspirin, Plavix, heparin or warfarin) often results in improvement – sometimes the abolition of the migraine.
Very low platelet counts can result in dangerous bleeding – indeed, Hughes Syndrome is one of the few situations that can perversely lead both to clotting and to bleeding.
My own talk this year is on an interesting question that is “are there circumstances other than thrombosis where anticoagulation might be beneficial?”. Today’s ‘patient of the month’ could be one such example.
However, where a Hughes Syndrome patient on warfarin does develop worrying features, and INR testing is not close at hand, we have found than an injection of heparin (it must be the low molecular weight variety) is safe, and often an effective short-term measure prior to re-aligning the warfarin dose. Obviously, this could only be under the guidance of the patient’s doctor.
One colleague whose work particularly impressed me was Tom Greco. Tom and his wife, practising in a clinic in Waterbury, Massachusetts have built up a large clinical practice of patients with Hughes Syndrome. They are strong believers in the frequency of the syndrome, and find that many current publications under-estimate its prevalence.
My memories of the Hammersmith are still vivid – for it was here that we described the antiphospholipid syndrome. The ward rounds, the animated discussions of our cases, the presentation of our findings to the ‘grand rounds’ – and, in 1982, the major presentation of the syndrome to the ‘Heberden Round’ – the annual meeting of the British Society of Rheumatology.
Firstly, that Hughes Syndrome, if it starves the brain of oxygen, can mimic almost any neurological condition. Secondly, that warfarin under-treatment in some cases of Hughes Syndrome is a waste of time. Rather like treating an under-active thyroid with half the dose of thyroxine, or treating diabetes with half the dose of insulin.