The second warmest November on record! Another record! (at least here in the south). This time last year we were under a (record?) heavy snow fall.
November brings the annual American College of Rheumatology (ACR) meeting. Far and away the best international meeting in our field, especially for those working in lupus. I have not been for three years, but this year was invited to run two consecutive ‘meet the professor’ breakfast sessions. These sessions are limited to thirty or so pre-booked attendees, and cover dozens of topics. I’m pleased to say that the ‘antiphospholipid’ session was fully booked. It was also very encouraging to see that the first nineteen papers in this massive meeting were all on our syndrome. Interest is spreading!
The other November meeting was in York, arranged by a group called the Primary Care Rheumatology Society. This is a group of GPs who have a special interest in rheumatology. I gave a Friday lecture on ‘the big three’ (lupus, Sjogren’s syndrome and Hughes syndrome) adding, I hope, to the spread of awareness of these conditions. For me it was a thoroughly enjoyable day (I had never previously visited this beautiful city).
Case of the Month
Mrs GJ, aged 41, had been referred by a physician colleague as a possible case of Hughes syndrome. She had had one suspected (but not proved) leg clot (DVT) in the past, and had a long history of headaches, balance problems and ‘pins and needles’.
Her sister had suggested that she be tested for Hughes syndrome, and the tests did, in fact, turn up a positive anticardiolipin result (aCL). All other tests were essentially negative, and an ‘unproven’ diagnosis resulted. She was started on low dose aspirin. Some nine months later (despite some improvement in the headaches) she developed a more definite DVT and was started on warfarin.
She was investigated for a range of clotting disorders and, once again, the only positive test was a medium-to-strong positive anticardiolipin (aCL) result.
Her family history (once again) was interesting. She had two sisters with multiple sclerosis (MS), another with migraine, and a fourth sister with Hughes syndrome.
What is this patient teaching us?
Once again, the family history can be important in diagnosis. As well as a sister with Hughes syndrome and another with migraine, she had two other sisters with MS. For me, the link (or relationship) between Hughes syndrome and MS is tantalising. Some years ago, we ran a questionnaire in our clinic at St Thomas’ Hospital. We asked the question ‘did your doctor at any time consider your diagnosis to be MS?’ The result was striking - over 30% of patients with positive antiphospholipid tests replied ‘yes’ – compared with 8% of the remainder of (mainly lupus) patients.
I believe that there are three possible reasons for the observation:
1.That the two conditions, with balance and visual disturbances, sensation loss and other ‘neurological’ features can be hard to distinguish (my own preferred explanation).
2.That MS – also considered an autoimmune disease – is one of the spectrum of diseases (such as low thyroid) seen in ‘autoimmune families’.
3.That MS, like, for example, arthritis, is made up of a number of causes, each targeting the nervous system.
Given the importance of MS, to me it is very surprising that still, thirty years after our description of the antiphospholipid syndrome, there are few good studies of this conundrum.