- October 2011
A lovely autumn – still dry in the South East.
It’s hard to believe that the London Lupus Centre has been going for five years! We estimate that we have now seen lupus and Hughes syndrome patients from 72 countries! Working here at London Bridge Hospital is a treat – overlooking the pool of London, between London Bridge and Tower Bridge, the area around the hospital, with the new ‘Shard’ skyscraper, Southwark cathedral, Borough market, and London Bridge station, has a real ‘buzz’ about it. Of course, we also have the London Dungeon and the ‘London Experience’ nearby, sometimes with ghosts, skeletons and ‘dead bodies’ out on the pavements – not a particularly good advert for our hospital!
Our annual ‘Ten Topics in Rheumatology’ is spreading worldwide, with ‘Ten Topics’ meetings now held in Barcelona, Nice, Rome and the Far East (Manilla last year, Hong Kong this year). This month ‘Ten Topics’ were held in Beirut (with Chris Edwards from Southampton and our unit lecturing, and Argentina (with Laura Bertolaccini and Maria Cuadrado taking part); these two meetings had 200 and 800 attendees respectively. I like to think that the Ten Topics meetings have contributed to teaching about lupus and about Hughes syndrome.
Case of the Month
Mrs K U, aged 41, gave a three year history of ‘chronic fatigue syndrome’ as well as aches and pains, and a tendency to ‘irritable bowel’. An initial diagnosis of ‘fibromyalgia’ (fatigues and aches and pains) was made.
A year later, in 2009, she developed slight shortness of breath, dry mouth, and frequent headaches. The headache became more severe, and frequently migrainous, so much so that on one occasion she was admitted to hospital in ‘status migrainosus’. A brain MRI was normal as was a chest x-ray. Subsequent investigations showed a positive ANA (anti-nuclear antibody) and a positive anticardiolipin test (strongly positive).
Clinical examination did not reveal any cause for the shortness of breath, but she did have two important critical signs. Firstly, she had a bone-dry Schirmers test (a simple test in which a strip of standardised blotting paper is hooked over the lower eyelid). The blotter is usually soaked in seconds. In Sjogrens syndrome (see below) it can remain dry for the full five minutes of the test.
Secondly, she had prominent livedo reticularis (blotchy ‘corned beef’ skin) on the forearms and thighs.
She was started on Plaquenil with (after a month or so) improvement in her aches, pains and fatigue.
The headaches remained. Aspirin failed to help. She suffered a mini stroke despite being on aspirin. A three week trial of heparin helped to improve the headaches.
She was ultimately started, in 2010, on warfarin, keeping the INR high at 3.5-4.
Six months later, she was a ‘changed person’. Not only had the headaches gone but, interestingly, the blotchy livedo skin had improved. As an added bonus, both the shortness of breath and the ‘irritable bowel’ had improved with the starting of warfarin anticoagulation.
What is this patient teaching us?
The story of this patient is a recurring theme: the association of Hughes syndrome (migraine, stroke and fatigue) with Sjogrens syndrome (dry eyes and mouth, aches and pains, positive ANA).
The improvement of patients with this clinical combination after starting Plaquenil and anticoagulation is often striking. The disappearance of the livedo is something we often see following successful warfarin anticoagulation.
In this patient, the shortness of breath improved (it is conceivable that this patient was suffering from mini lung clots). More surprisingly, the ‘irritable bowel’ symptoms immediately improved. A coincidence? I have certainly seen irritable bowel symptoms in a small number of our Hughes syndrome patients improve with anticoagulation. One possible explanation is that ‘bowel ischaemia’ (a sludgy blood supply to the bowel) might be contributing.
Finally, any clues to the cause(s) of Hughes syndrome? Yes. Two clues – ‘genetic’ and ‘enviromental’. Mrs K U had a strong family history of autoimmune disease (thyroid and rheumatoid in her family).
Secondly, her history possibly went back over twenty years. At the age of 21, she went down with severe glandular fever. She was laid up for over a month. She did not completely recover, fatigue, aches and pains and intermittent headaches, dating precisely from the glandular fever, continued for several years, before easing up, only to return in her late 30s.
Although final proof of a link between some cases of glandular fever (E-B virus) and autoimmune disease such as Sjogrens and Hughes syndrome remains elusive, the clinical suspicion is strong.