Sticky Blood-Hughes Syndrome Support

Prof Graham Hughes' April blog

A wet bank holiday. An ‘indoors’ day. Shopping? No. Go to watch Charlton Athletic (my wife’s favourite team)? Neither of us keen to get wet and cold. So, an April blog.

An interesting paper was published recently in the journal Rheumatology. In a collaborative study from eight centres in Italy, 959 lupus patients were divided into two groups – those who had suffered neuropsychiatric involvements and those without.

The two groups were then compared to assess whether any clinical or laboratory features cropped up more frequently in those with neurological involvement.

The findings were largely negative, but with one striking exception: antiphospholipid antibodies (aPL) were strongly associated (p<0.0001). Not surprisingly, stroke, in particular, correlated strongly.1

Over the years many groups, including my own team, have studied the effects of different antibodies on the brain. A number have had the spotlight on them, but hopes that they would prove diagnostically useful in diseases such as lupus have proved short-lived.

In 1983, the description of the antiphospholipid syndrome – related to, but separate from lupus, highlighted the importance of cerebral disease as a feature of the syndrome.2 As the years have gone by, the clinical links between APS/Hughes syndrome and the world of neurology have become stronger and stronger.

Not surprising therefore that those patients with lupus who are aPL positive (about 30-35%) have been found to have more neurological disease.

Clinically, these observations have important implications for the treatment of lupus.

When a patient is admitted to hospital with ‘cerebral lupus’, treatment is, rightly, aggressive, usually with steroids and immunosuppressive drugs. But what about the role of aPL and clotting?

In the parallel world of APS, the introduction of treatment (such as heparin) in patients with neuropsychiatric illness can be very, very striking, as in the following case of the month …

Case of the Month: ‘A weekend drama’

Mrs DP, a 54 year old nurse working in a GP group practice, was admitted to London Bridge hospital by the cardiologists with angina and a suspected heart attack. She had suffered from shortness of breath and chest pain for several months, and on admission, the tests showed possible myocarditis.

However, the picture became more complicated.

She had complained of increasingly severe headaches, ‘behaviour difficulties’ and very peculiar ‘seizures’. The level of consciousness fluctuated. A brain MRI showed 8-9 small ‘dots’ but nothing more widespread. Among the other tests run by the cardiologist was a blood test for aPL.


So, late on Friday afternoon, I was asked to see the patient, as a possible case of APS. Certainly the odd cerebral and cardiac features could fit. So too could the MRI. She also had skin livedo (‘corned beef skin’). The GPs in her workplace confirmed the complexity of this patient’s history.

We decided to start her immediately on heparin – (low molecular weight ‘Fragmin’ by injection twice daily).

On Sunday morning the cardiologist called me at home. The patient was well, sitting up in bed with a cup of tea and chatting to her family!

Over the next few days the story became clearer. The patient, an excellent historian, recalled a lifetime tendency to headache and migraine, worse over the past year, worsening memory loss and bout of confusion, odd seizures, many witnessed by her husband, and slowly worsening chest pains and shortness of breath.

Now all better! In the words of one colleague: ‘a life-changing diagnosis’.

What is this patient teaching us?

Many lessons. Firstly, that Hughes syndrome can and does affect the brain.3 I passionately believe that one day the syndrome will be recognised as an important chapter in the neurology textbook.

Secondly, that despite the severity and the long history in this patient’s case, the response to anticoagulation can be immediate.

Thirdly, the syndrome of ‘sticky blood’ can deprive other organs of oxygen – in this patient’s case both the heart and the skin (livedo) were also clinically affected.

Finally, where next? As few weeks longer on heparin then almost certainly on to warfarin, with an INR in the high 3 range.

And we will be testing her relatives, a number of who have histories of seizures and migraine.


1.Govoni et al. Factors and comorbidities associated with first neuropsychiatric event in systemic lupus erythematosus: does a risk profile exist? A large multicentre retrospective cross-sectional study on 959 Italian patients. Rheumatology 2012 51: 157-168

2.Hughes GRV. Thrombosis, abortion, cerebral disease and the lupus anticoagulant. British Medical Journal 1983 287: 1088-9

3.Hughes GRV. Understanding Hughes syndrome: case studies for patients. Springer ISBN 978-1-84800-375-0

13 Replies

I was diagnosed by a neurologist (a Doctor of Osteopath-DO, not an MD). He had just come back from a conference somewhere and knew the symtpoms and being a DO went over my complete health history which sounds a lot like patient above and well, the rest is history!! This was in beginning of 2008.

I think maybe we need to start targeting some of the larger Neurology conferences sometime in the near future?


This is great - I have just forwarded it to my GP and also my children's paediatrician! Need I say more! Mary F


Thank you for sharing! In Canada hardly anyone has even heard of APS. I don't have a lot of time as a mom and full time employee but I want to raise awareness in Canada. I am looking into raising awareness through Canada's Heart & Stroke Foundation. I am trying to find a local contact. I am participating in an event this June. :)


Great news, and of course there are a host of blogs form Professor H, and of course medical papers attached to the Hughes Syndrome Foundation etc, best of luck with this... we will all change the world etc etc Mary F x


I have been referred to a neurologist and awaiting appointment. I am aPL positive. I have had increasing neurological symptoms the last couple of years but my INR is usually high 3's or low 4's. It will be interesting to see what the neurologist makes of my history and what he/she suggests in terms of help and treatment.

Is anticoagulation meant to stop these neurological problems or just help reduce the severity or how quickly they progress?

Also, are there any differences between warfarin therapy and Fragmin/Clexane therapy in helping with the neurological side of things in APS?


Hi Tavelnut, I have the neuro symptoms and my aCL titre was 22 so no diagnosis or treatment but have many APS symptoms. What was your aCL titer? Has the anticoagulant therapy eased your symptoms? What country are you getting treated? Is your doctor in agreement with doctor Hughes' theory that many of us are misdiagnosed with MS? Sorry, lots of questions...might as well get right to the point lol.


I don't know what the actual amount was but I have tested positive for lupus anticoagulant on every visit to the hospital. I am in the UK. Way back in 2003 I had surgery and had a bad reaction to it and the docs then thought I had MS (this was before I was diagnosed with APS in 2010) so it has come up as a possibility but I have never been formally diagnosed as having MS. I am on warfarin between 3.5-4.0 INR but it is rather unstable so I have to test weekly. I think the warfarin has helped to prevent further DVTs and PEs along with the filter I had inserted a couple of years ago. However, I don't think it has done much for the fatigue/tiredness and other symptoms such as brain fog and some of the neural problems. In addition I've developed hearing loss and loss of field of vision in last two years which has been put down to the APS and that is whilst I'm on anticoagulation therapy.


Thankyou Travelnut for your reply with valuable info. I too suffer extreme attacks of fatigue and some loss of vision which they dx as glaucoma, several bouts of phlebitis and miscarriages, migraine auras,neurological problems,balance and gait, livedo reticularis,vertigo, raynauds and ringing in the ears etc. Being a lab technologist I questioned the dx of MS because of getting diagnosed with several diseases at once.....too fishy for me. But I was hoping you were going to say that being on warfarin eased up on the symptoms. I live in Canada near Niagara Falls so not too far from US and am trying to find a well informed Doc who would be up to speed on APS not like the ones who have been more than happy to see my back out their Wish I lived in the UK right now. I'd love to sit face to face with Graham Hughes. It would probably only take a minute for him to shake his head and wonder why I'm not being treated agressively. A specialist in Poland did tell me that I have APS but I wasn't in the country long enough to treat me. Have you ever been treated with steroids? Let's all pray for a breakthrough. My intuition is telling me that they will find something to block the inflammatory pathway...hoping


That is very interesting, and helpful to me. I had a stroke a few years ago and was diagnosed as having APS. I have a number of congnitive/ brain issues, but not sure if it is down to the stroke or APS (they are certainly worse some days than others).

My current Hematologist says that she can only comment on my INR and that there is NO evidence that APS symptoms are worse when the INR is low. I pointed out that I usually know what my INR is by how I feel, but don't think she believed me. I am hoping to be referred to St Thomas's so I can have some answers, as I'm feeling really frustrated at the moment!


hi, DelH,

As it's you going through this , only you know what you feel like at the time.

The hemotologist does not take these things into account when making decisions regarding their treatment of you.

IMO you have to make these things clear to them and stress that it's how you feel right now, after all, it's you living with these symptoms and not them.

Hope you get the answers you're looking for and some relief as well.

Wayne L


anyone have a personal email for dr.huges.... really need to consult him on my wifes cronic aps.... its been quite a year... and the doc's here in Canada are not getting any answers, to her ongoing issues


Hi Skylark - Prof Hughes can only be contacted through the London Lupus Centre at London Bridge Hospital: This is a private clinic not part of the UK's NHS which means overseas patients can be treated there.

Please note that the charity is completely separate from the London Lupus Centre.


I have had all the symptoms of Hughes syndrome for years.

DVT, Miscarriages, Low birth weight babies, Epilepsy, Heart attack.

Had been seeing a neurologist for years ( don't feel he needs a capital N)

My question is what training to they have !


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