New research reveals more about Giant Cell Arteritis

[Suzym2u]

New research has been revealed about how Giant Cell Arteritis (GCA) may be caused by a certain group of white blood cells called "neutrophils". GCA (also known as Temporal Arteritis TA ) is a condition which causes inflammation in the blood vessels and primarily affects the elderly.

vasculitis.org.uk/news/rese...

[PMRpro]

Thank you Suzy - though given the number of people in their 50s I know with GCA I'd dispute the use of the word "elderly". I'll accept "older patients"

[Suzym2u Moderator Vasculitis UK]

I agree with you, but they used the word elderly in the paper as I understand the average age of onset is 72 years. (My husband is just 70 and no way does he see himself as elderly at all.)

The books/medical profession tell us that the commonest age of the onset of Wegener's Granulomatosis (GPA) is between 60 and 70 years of age. I know far more people with WG below the age of 50 than I do above that age. The average age on the VUK mailing list for being diagnosed with Vasculitis , was 52 years. That was about 3/4 years ago.

all the best

Susan

[Hidden]

You can access the Journal that the article appears in online for free ( google Circulation research journal ).

It is very technical ( I didn't understand most of it! ) but it has a handy section at the end which details the implications.

Unfortunately ( from my perspective ) it doesn't appear to aid dagnosis but does help with disease monitoring. I wonder if it would aid those with " normal " ESR and CRP who are still symptomatic.

The 6 months of steroid bit appears to come from the fact that the last blood samples they took were at 24 weeks, not that GCA always disappeared then and treatment could stop!

[Jane1175]

I'm pleased to hear about the research going on for gca. I was diagnosed with polymyalgia jan 2013 put on 20mg steroid ,got down to 4mg then in October got gca put on 60mg came down to 35mg but now back to 50mg, Im 55 years young(ha,ha) .I don't like the steroid one bit because of the side effects but my biggest fear is loosing my sight, I also had a mini stroke when I got gca. I hope they one day come up with a drug to treat the illnesses that need steroids with a drug with less harmful side effects.

[maisie5]

Thanks for this suzy. I was diagnosed with Gca in 2009 and have been treated with high dose preds and methotrexate ever since. Every time I have weaned off the preds I have spent only 6 or 7 weeks in remission before relapsing totally. I have had all the typical symptoms ie. headaches, jaw pain etc. What I find really interesting about the research is the six month alterations in white blood cells. My first relapse was after approx. six months but my ESR was only slightly raised and all other blood results normal. I lost my eye sight twice in hospital but only for a few minutes each time thank goodness. I am now down to 6mgs pred daily but am now having ear ache and hearing loss for which Addenbroo

[maisie5]

(continuing from above) have given me a hearing aid. These audio symptoms are in addition to my GCA symptoms. Does anyone know of a connection between GCA and other vasculitis diseases?

[BronteM in reply to maisie5]

Hi Maisie, I was originally told that as GCA is a large vessel Arteritis it overlaps with Takayasu's Arteritis. Some specialists like to say that you have GCA if you're over 50 and Takayasu's if you are under fifty. This seems to be changing....I am now officially a Takayasu's patient even though I am over fifty. In my case I had all the TAK symptoms, and few GCA ones, so my diagnosis has gradually changed. There is also an idea, I think, that GCA and Takayasu's are on a sort off continuum and it is hard to tell where one starts and the other finishes. When in doubt I say that I've got large vessel vasculitis, which covers everything!

[maisie5 in reply to BronteM]

Thanks for your info BronteM. I am 61 yrs old and don't mind what label they give me as long as they find something to stop the relapses! What finally made them change your diagnosis? I have also been diagnosed with a aortic anyeurism which doesn't fit with Gca or does it?

Regards, M5.

[BronteM in reply to maisie5]

That's interesting...I thought aortic problems were definitely an indication of Takayasu's? I shifted that way because I had no pulses in either arm, wonky blood pressure and a clear temporal biopsy. Ultra sound scans show problems in the arteries in my arms and neck. A PET scan showed inflammation in my aorta....in fact I ticked off all the TAK criteria except age. But the treatment is the same for both, so not a big deal really. Hope you get sorted soon - not sure why a firm diagnosis is good - but it is!

[maisie5 in reply to BronteM]

Thanks for your interest BronteM. Always good to know how others are coping.

M5.

[Hidden in reply to maisie5]

Hi Maisie5

Interesting discussion. My understanding was that it was known that GCA could cause aortic problems and anureysms in a small number of patients.

They have found aortic inflammation in post mortems of patients with GCA. The vexed question is whether to screen for this complication. The thinking was not before as the average age of GCA patients was 70 odds, no point in screening for something that you couldn't offer a fix for.

As more younger patients with GCA are now being diagnosed that might well change.

Best wishes

Keyes

[PMRpro in reply to maisie5]

It is one of the things that should be screened for every couple of years in patients with or who have had GCA - there is an increased risk. It is mentioned in the BAR Guidelines for diagnosis and treatment of GCA.

This is actually the case for most large vessel vasculitides - and there are 3 sort of levels of this PMR (which is the baby brother), generalised GCA and temporal arteritis. It is possible to have GCA in any artery with an elastic part to the artery wall. It is probably present in the arteries in the chest in many patients who are just told they have GCA when it gets to the temporal artery. I have PMR (officially) but it started with thigh and upper arm claudication - not recognised by the docs but a clear sign of GCA. I even had jaw and scalp pain but I was very lucky and it disappeared.

[PMRpro]

"as the average age of GCA patients was 70 odds, no point in screening for something that you couldn't offer a fix for"

There are fixes and prevention at that stage is far better than it getting worse unnoticed and having a patient with a burst AAA admitted to A&E.

As for the "Average age is 72" - since half of patients in their late 70s and 80s have PMR and a lot of them at that age go on to develop GCA there need to be quite a few patients who are UNDER 72 to make that average. Unfortunately most medics aren't very good at that sort of maths!

On another forum we have 2 women in their 50s definitely with GCA and several more in their early 60s. Last year a man of 37 in South Wales was found at post mortem to have died of a stroke due to untreated GCA - he'd presented with typical GCA symptoms but wasn't investigated as he was "too young". One lady I know has a friend whose teenage son has been diagnosed with GCA. And it is GCA - confirmed by finding the cells.

The best bit of this piece of very basic ground level research is that they have a handle in these cells that makes it possible to monitor the treatment better and means they should make it possible to not reduce the pred dose when it isn't appropriate - as has happened for too many people I know. Because their doctor is so desperate to get them off pred they reduce too fast and too far - and the result is a flare meaning having to go back higher than they would have done had they proceeded more slowly. These cells appear to be specific to GCA and TA forms of vasculitis (unlike ESR and CRP which go up with a cold, flu and pregnancy amongst other things!) and may also be present in other forms of vasculitis.

The other bit - and that will take a lot longer to realise - is that they can look now for drugs which inhibit these cells which could form a proper treatment. That, however, is likely to take years probably, not just months. For the present the most likely effect is that GCA patients will be on a higher dose of pred for longer.

No - the 6 months didn't come from the 24 weeks of monitoring, it is in the introduction and the discussion of the paper which I have read (and I do understand most of it as I'm a medical biology scientist but in a different field). I have no idea where they got the figure from - they refer to the British Association of Rheumatologists paper as source but that says categorically that you need at least 5 months at above 20mg and the taper is over at least 2 years, using the words "several months" - so not what they say here which is:

"GCA is initially treated with a six-month course of high-dose steroids."

and

"It is commonplace for patients to come off therapy after six months but our research shows this may be too early for many."

which are direct quotes. And neither is correct - "several" is more than six in this case.

I just hope that that isn't the bit patients and doctors take from the press release when they read it - because it will just get people's hopes up and encourage lazy doctors who don't do their reading properly. I hope it is the "six months ... may be too early for many" they remember and they stop trying to force patients to reduce too fast.

[Hidden]

Giant Cell Arteritis is only called 'Temporal Arteritis' when it specifically affects the temporal arteries not when it affects other medium and large arteries of the body. People over the age of 50 are hardly considered 'elderly' when the average life span of women in the UK is now 82.5 years! 100 years ago, 50 might have been elderly, but not these days!

[Hidden]

The word 'older' is meaningless unless one can state 'older than ....?'

The average age of onset is also statistically meaningless unless one explains how one arrives at this figure. There are all kinds of ways of working out averages. Medically, it is unhelpful to state that there is an 'average' age when the age range spans from approx 50 to approx 100 years of age.

Unfortunately, referring to patients as 'elderly' excuses doctors from being dismissive as all 'elderly' people have 'aches' and 'pains', find it an effort to bend down, etc.

Still more confusion being produced and most serious concerns being ignored! Many patients with GCA are not offered a stroke assessment which is bizarre (and shocking) considering how much one can help oneself to reduce risk if the risk is high (of course when the arteries are inflamed it is higher than it would be otherwise). Not only eye sight but also hearing can be seriously affected with GCA as well as other arteries in the body, which can become blocked and damaged and place vital organs in danger.

Prednisolone itself (a glucocorticosteroid) bumps up sugars so diabetic checks need to be made when taking it and it can cause osteoporosis so Calcium and D3 might be needed. Not only diabetes but high blood pressure can also be a problem.

The treatment is the same for patients who undergo a temporal artery biopsy as for those who refuse to have it. Many don't want to have an inch removed from their cranial artery to be put through for a new drug trial, for which doctors get paid and drug companies make lots of money. Doctors don't inform patients of their rights though it is illgal not to do so.

Non invasive scans are available, and patients should request them. The more patients are informed, the more doctors will realise that they cannot pull the wool over the eyes of patients with this serious, life threatening or incapacitating illness.

Most concerning recently I heard of a GCA patient who died because she was given a drug in A & E which restricted blood flow. If you are offered another drug for another health matter, beware!

[Hidden]

Hope they find the cause soon and a more consistent treatment regimen. It's very hit and miss at the moment and if you have a caring and responsible doctor, you're very lucky, neutrophils or no neutrophils!