Prof Hughes' blog August 2011

Last day of August. Grey and cold. “The coldest summer for eighteen years” – how we British love our records!

Earlier this month, I gave two lectures in Colombia, South America – two days in transit, two days in Colombia. What a wonderful reception – several hundred young doctors, keen and hungry for information. One told me that they have an ‘epidemic’ both of lupus and Hughes Syndrome – now two of the most commonest illnesses they face.

It was especially good to meet with two friends – Herman Gonzalez who had been a visiting fellow in the Louise Coote Lupus Unit at St Thomas’ 25 years previously, and Antonio Inglesias, Professor of Rheumatology in Colombia who has produced a massive two-volume history of lupus – a masterpiece.

The closing dinner on the evening of my lecture was also memorable. A major football match was being played that evening between Colombia and Mexico. A giant TV screen was erected, and we were all given yellow shirts and caps (Colombia). Colombia lost, but still great fun.

August also brought a new role. I was invited to become a patron of Britain’s national arthritis charity, Arthritis Research UK. I met their team, led by their new chief executive Liam O’Toole and also their director of philanthropy, Jacqui Manning. A real sense of optimism and purpose. I have no doubt that the charity will thrive, and break out of its old ‘clubby’ reputation. I will certainly do my best to help.

Finally, Kate Hindle gets married this Friday. All of us here wish her every success and happiness.

Case of the Month

Mrs AL, a 50 year old lady from Plymouth, was referred to the London Lupus Centre for a second opinion. Her history was complex. For some years she has suffered circulation problems, with two DVTs, one documented pulmonary emobolus, migraine, angina, transient ischaemic attacks (TIAs) and finally a slight stroke. She had also, in her twenties, suffered three miscarriages.

Also, for a number of years, she had suffered from widespread muscle and joint pains and aches. One physician had diagnosed fibromyalgia, which seemed to fit the pattern of her symptoms.

Her family history (important) was interesting. Her father had suffered autoimmune haemolyptic anemia, migraine and an early stroke. Her daughter had had five miscarriages but not been tested for antiphospholipid antibodies (aPL).

On examination, apart from evidence of slight residual weakness from the stroke, there were no abnormal findings in Mrs AL – with one notable exception. Testing her eyes for dryness (the so-called Schirmer tear test) showed a complete lack of tears – a finding suggesting Sjogren’s Syndrome.

What is this patient teaching us?

Clinically, a diagnosis of Hughes Syndrome is fairly clear – a diagnosis confirmed by the finding of strongly positive tests for aPL. She has been started on warfarin.

The history of ‘fibromyalgia’ is interesting. Although it is a common diagnosis, it can be misleading. For example, many of my lupus and Sjogren’s patients have initially been diagnosed with this label. In Mrs AL’s case, the diagnosis is almost certainly Sjogren’s Syndrome (dry eyes, dry mouth and aches and pains) – a syndrome which often goes hand in hand with ‘sticky blood’.

Normally in these blogs, there is a conclusion to the story. Not yet with Mrs AL. She has been started on Plaquenil for the Sjogren’s aches and pains and warfarin for the Hughes Syndrome. I promise to give an update in a few months.

2 Replies

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  • Hello Dr Hughes!

    I am in a flare now and seeing a new (to the area) rheumatologist very soon. Concern is that I have sjogrens as well, and they will be doing tests for this. I have very dry, itchy, red eyes and swollen salivary glands.

    She will be doing a full workup but already have had APS titers come back elevated (they were undetectable when I was in London last September).

    Thought is I got a virus at work or something as also have high fever intermittent (always elevated lately), and swollen lymph nodes in neck.

    Don't worry- I am in good hands and following the doctors advice. Am still able to work and do my school work, but very tired at times.

    Hoping to see you on next visit to UK (planning on spending a few months there beginning May 2012).

    Keep up all the great work and please keep me posted on research!

    Kristina

    PS- My cardiologist is prescribing a LOT of Pradaxa (dagibatran) for non vavular AFib and transitioning all those patients off warfarin with great results. I may ask if I can do some small study of side effects, quality of life, cost, etc. using his patient base.... Would love some help on this! :)

  • Dear Dr. Hughes,

    I live in New York. I have von willebrands low factor VIII, and a diagnosis of APS. The drs do not know how to trat me. I was 42 when I had a stroke. I am also diagnosed with small vessel lacunar disease and atherosclerosis. I am on a baby aspirin. Do you have any suggestions for me, or know of a good dr in the US that i could see? I was on Plavix but i hemorrhaged. Thank you for your time and your work. Teresa

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