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Professor Graham RV Hughes Blog: March 2018

March 2018

01 March 2018 by Professor Graham R V Hughes MD FRCP

Wet, wet, wet. March this year, at least here in the South-East, has been dreadful – rain, cold winds, snow and most of all, grey skies. I swear that we have had less than 10 hours of sun this month.

I hate to think what the nation’s average Vit D level is this year?

For me, however, there was good news.

Our new website (ghic.world) has now come on stream. Free to subscribers, the website aims to link doctors and patients dealing with Lupus and Hughes Syndrome in a global network.

We have had a flying start, with 78 international doctors on board. Our main goal is to educate and we plan to run a series of regular features. These include:

News from Medical Journals (including the free uploading of 2 articles from each issue of the LUPUS journal, thanks to the support of Jovie McMillan of Sage Publishers)

“And now for something different”

Prinzmetal’s Angina

Mrs K.I., aged 59, had been investigated by the cardiologists for angina and heart attack. The attacks of chest pain had been happening for a couple of years. Mrs K.I. underwent tests in a cardiology clinic. Blood tests (including cholesterol) and blood pressure were normal, but the cardiac MRI showed patchy areas of poor blood flow. A coronary angiogram also showed ischaemia, but, interestingly, the coronary arteries themselves were normal – a condition which was labelled “Prinzmetal angina”. (This form of angina was report by Dr Prinzmetal, former Professor of Cardiology at U.C.L.A. in America). More recently the condition was also described as ‘Syndrome X’.

In simple terms, it is ‘angina with normal coronary arteries’.

Her past history was one of frequent migraine headaches, and endometriosis, treated with a form of HRT (which the cardiologist stopped).

Her pregnancy history was complicated by 4 early miscarriages.

In the family, her mother had Hashimotos Syndrome (low thyroid) and her brother had suffered a DVT. She was referred to the London Lupus Clinic. In addition to the above, she complained of aches and pains, irritable eyes (which tested dry on Shirmer’s ‘blotting paper’ tear test).

And yes, as the cardiologist suspected, she tested moderately strongly positive for antiphospholipid antibodies (she also had a positive ANA of 1 in 360).

To cut a long story short, she was ultimately treated with warfarin. Angina gone, headaches largely gone.

What is this patient teachings us?

In the 35 years since the description of the antiphospholipid syndrome, the focus has been heavily on obstetrics and on the brain and its many presentations in Hughes Syndrome.

However, the heart is also clearly vulnerable. Heart valve problems have been recognised for years, but angina – obviously a candidate for ‘sticky blood’ – has had less exposure – at least until the past 15 – 20 years. There are now a number of very good studies, notably of Dr T P Greco in Connecticut, Dr Urbanus and his group in Holland and Professor Meroni and colleagues in Italy.

In 2002, our group reported a small series of Hughes Syndrome patients with angina – but with normal coronaries (Syndrome X). Now it turns out that Syndrome X and Prinzmetal’s angina are one and the same thing. It doesn’t require much head scratching to guess that there are a significant number of angina patients (especially women under 45) who have the very treatable condition, ‘sticky blood’ or Hughes Syndrome underlying their chest pains.

As always, it depends very much on clinical suspicion (migraine, miscarriage, family history), and simple blood testing (aPL/antiphospholipid antibodies).

Time will tell.

Professor Graham R V Hughes MD FRCP

PROFESSOR GRAHAM R V HUGHES MD FRCP

Head of The London Lupus Centre

London Bridge Hospital

Source: ghic.world/self-help/blog/m...

5 Replies
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Thanks so much for posting, I found it really interesting.

I have suffered with chest pain and even full on heart attack symptoms, chest pains going down left arm, up neck and into jaw, with numb and tingling limbs and lips etc.

The doctors and cardiologists I've seen have never even considered Syndrome X, even though they know I have been diagnosed with Chronic Thromboembolic Disease since 2005, so definitely have sticky blood.

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My advice is to seek a specialist in the UK. Not all rheumatologists understand Hughes/APS.

With good wishes,

Ros

1 like
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My EDS rheumatologist has asked my GP to refer me to Prof DCruz at St Thomas Hospital, so fingers crossed he will finally be able to sort me out! 😵

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Excellent. He would be my recommendation. Dr David D'Cruz has been trained by Prof. Graham RV Hughes after whom Hughes Syndrome/APS is named! Hopefully, as a new patient, you will be seen within the recommended period of time.

Do let me know how you get on!

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Yes hope so too , I will definitely let you know how it went on after my appointment😊

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