Professor Graham Hughes' Monthly Blog April 2017

BLOG – April 2017

Big news! A group of patients and doctors here have set up a new charity, whose main purpose is education. Specifically, its aim is to provide a website which links those suffering from Hughes syndrome and those involved in its treatment. We know that many countries already have both patient groups and medical research groups – our aim is to globally link as many of these groups as we can.

The charity, named GHIC (Graham Hughes International Charity) is free of charge and will offer regular features, including clinical updates, ‘Patient of the Month’, Q&A’s, review articles, and news from the conferences.

In addition, the publishers of the international journal LUPUS have agreed that we can publish excerpts from articles dealing with the syndrome.

With our international team of medical experts, we trust that the charity, with its focused remit of global education, will add to the work of other charities and patient charities worldwide.

Patient of the Month

“A Perfect Storm”

The more I see of patients with Hughes syndrome, I feel that it is, first and foremost, a ‘neurological’ condition – indeed in some patients, it seems that all parts of the nervous system – the brain, the spinal cord, the nerves, and even the ‘internal’ nerves – the so-called autonomic nervous system are involved. This was certainly the case with Mrs L.T.


Mrs L.T. a 46-year-old housewife made the journey from Atlanta, USA for a second opinion. I saw her with my colleague, Natasha Jordan, who listed her history as follows:


1. In infancy - ITP (‘idiopathic’ thrombocytopenia purpura) (low platelets)

* Migraines, possible seizures

2. Teenage onwards - Hallucinating music, olfactory symptoms

3. Pregnancy - Recurrent 1st trimester pregnancy loss

4. 2012 onwards - Partial complex seizures

* Visual disturbance

* Transient ischemic attack

* Possible transverse myelitis

* ‘Dysautonomia’

5. Upper limb DVT

Blood Tests

Low level position IgG anticardiolipin antibodies

Low level positive IgA anti-Beta 2 GP1antibodies

Negative lupus and thyroid tests

She had been diagnosed with primary antiphospholipid syndrome and started on Coumadin (Warfarin) - unfortunately, with no great improvement. Her INR ranged between 1.9 and 2.6.

What is this patient teaching us?

I thought it would be clearer if I numbered the problems, and addressed each separately:

1. ITP in Infancy

It is well known that ITP (low platelets) can occur in childhood and even infancy. It can also occur in Hughes syndrome and precede the main diagnosis by many years. A definite clue here.

2. Teenage

Migraines in Hughes syndrome often start in the teens. So also can seizures (see November 2016 blog). Interestingly, temporal lobe epilepsy seems, in my opinion, to come up a lot – ‘funny turns’, déjà vu, strange olfactory, symptoms all being features.

3. Recurrent pregnancy loss

In this patient, almost certainly related – think of what testing in pregnancy might have achieved.

4. 2012 Onwards

A dramatic escalation of the ‘brain storm’, mini strokes (TIA’s), visual disturbances, seizures, and now, possible autonomic nerve problems. To cap it all, development of spinal cord problems – “transverse myelitis” – something which we identified as a feature of the antiphospholipid syndrome in our original descriptions in the early 1980’s.

5. Upper limb DVT

As if we didn’t have enough to go on, an arm thrombosis.

6. Blood Tests

“Weakly positive” – here we have an example of conflict between the science of “Classification criteria”, and the coal-face of internal medicine. I think the diagnosis, based on largely clinical grounds, is clearly Hughes syndrome, despite the ‘borderline’ tests.


This patient was, correctly, started on Warfarin. The poor response? The dose is too low. Many patients with brain aspects of Hughes syndrome seem to need a higher INR, sometimes as high as 3.8 to 4. Indeed, many of our patients “know” precisely when their INR is too low.


We will keep you posted

Professor Graham R V Hughes MD FRCP

Head of The London Lupus Centre

London Bridge Hospital


29 Replies

  • Thank you Mary and prof Hughes!

    I believe that "brain aspects" in HS need a higher INR at around 4.0 with Warfarin (Coumadin). Exactly as prof Hughes says. That is also my personal experience.


  • Interesting read. Thank you. Cindy in NJ

  • Very interesting

  • This patient really could be me!

    I have spinal cord involvement as well as the other symptoms stated and I need an inr of 4 to remain well.

  • Glad you saw this, thought of you when I read it xx

  • I'm really chuffed with this! I've not related to many of the 'patient of the month' before and often feel alone with my issues. This has made me feel better knowing I'm 'normal' haha x

  • I know what you mean, my IgA levels are above range as well as LA but when I pointed this out to my consultant she was like - it's not related just one of those things.

    I see her tomorrow and feel like shoving it in her face!

  • Good luck with your appointment x

  • YEA!!!!

  • Count me in to join

    I always look forward to his patient of the month.Seems he takes time to evaluate the person with such care.Maybe it will be me one

    I love this forum

  • Thank you even more reasons why neurologist should get their act together

  • I should have said that not every Neurologist is bad, their is the occasional good one eg: Dr P Holmes Guys hospital

  • Nice to hear that, my daughter had a couple of good ones also. MaryF

  • Great link and info. A couple of points:

    1. Is there a link or do we just search for it?

    2. I have never displayed neurological symptoms, but when diagnosed, I was described to have "developed " APS. I also always had an INR of 2.0-3.0 (when on warfarin; now on Rivaroxaban). In fact I used to donate platelets in my teens and 20s...

    I have been referred to a haematologist (links to my post recently about which medical experts we "should" see) to give me more info on my APS and APS in general, alongside my host of other medical referrals (for the non-APS stuff).

    Thanks again.

  • Hi do you mean info on the new charity? In case you do, here it is, sorry for late reply, HU must have been working on platform this morning, as we had no notifications coming in


  • Thanks Mary - yes. Things dont always show in the clearest format on my phone (where I usually end up accessing posts from), so I wasn't sure if the Blog was a regular feature on HealthUnlocked (where I see the APS thread) and/or whether the charity was linked and I couldn't see it!


  • Hughes patient of the month " poster child"

  • Number 2. Teenage onwards - Hallucinating music. Interesting. Would love to hear more about that. Since high school (that I can remember) I've got music "playing".

  • Finally realised what was bugging me about this one - at the end it states the importance of diagnosis on clinical grounds (not just test results) but at the beginning thyroid and lupus are dismissed with a single line "negative tests".

    Now, I trust that since this is Prof Hughes a full set of thyroid tests, including antibody levels, will have been done together with a full review of symptoms against hypothyroid, before eliminating it - but I do wonder if the way it is written gives a false, possibly misleading impression, of the process, i.e. that for Hughes you need to look at the totality of clinical symptoms, history and test results, but for thyroid if the results from the lab (maybe TSH alone) say "in range" then it's not that...

    Is it just me (probably)?

  • Hi, unfortunately most labs, even if tests requested by GP's do very little, I had to go off myself and do very detailed thyroid tests to get a result, I did eventually pass the very limited NHS test for TSH and antibodies, and same with Hughes Syndrome and Lupus, although negative for years, I did then pass them all several times over. MaryF

  • Andrey are you sure you posted where you meant to? I see you are new. Please write a new "post" to ask your question if need be

    Welcome, Cindy in NJ

  • I am not sure if i wrote at the right place, i am first time here.Can you please guide me where should i write my question

  • I'll pm ( private message) you

  • Did this help, I wrote a post describing how to do a new post

  • Yes,thank you so much

  • Thank you for your time and your help.I actually just did wrote the message again and put the picture of my leg where the problem is.(

  • Glad I could help, welcome aboard!

  • I for one am thankful that I have a neurologist that is concerned about my APS but also pays attention to my neurological symptoms. I am still awaiting results of my cartoid ultrasound and additional black of work and waiting to get in for a follow up neck and head MRI. He is being very thorough and will be sending me to a Hematologist once the bloodwork is in. He seems to care about the APS and how it is affecting my migraines and numbness. I have had migraines since I was about 8 yrs old.

  • Hi Toddzchick,

    I understand that you found prof Hughes blog of April interesting.

    Many of us have had Warfarin which should help but the Doctor (probably not a Specialist of autoimmun illnesses as he did not understand that we have so very thick blood and need a high INR) did not dare to put her on the right level and she did not get better.

    I suggest you read his other blogs also. We can learn so much from eachother and from other patients stories described so well by the Prof Hughes.

    Prof Hughes also said that many patients know exactly when the Warfarin "kicks in" and the neurological symptoms disappear.

    Good Luck when yo see your Specialist very soon now!

    Kerstin in Stockholm

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