Takayasu or GCA.

[Caro12line]

Hi. I have been diagnosed with GCA of the Aorta. Can anyone tell me the difference between this and Takayasu? The signs, symptoms and treatment sound the same. Also GCA is always associated with PMR yet signs and symptoms are different. Except that if you have PMR there is a chance of getting GCA and vice versa. Can anyone clarify this please. Thanks

[orsen-trapp]

Some say if you're under 50 it's TAK and over 50 it's GCA. I'm 55 and definitely TAK, according to my rheumy. Treatment's the same anyway, I believe!

[Caro12line in reply to orsen-trapp]

Thanks orsen-trapp. How is your TAK monitored and managed? How do you know if you are flaring or suffering from side effects from the meds (Prednisolone)

[orsen-trapp in reply to Caro12line]

Very good question, Caro. I'm on Pred, tapering very slowly, with methotrexate to squash my immune system. As side effects of both and symptoms are very similar, the only way of checking if I'm flaring is by monthly blood tests, especially CRP and ESR. If those go up substantially I'll have another PET scan. The fatigue just hasn't gone away - spent most of yesterday in bed - and yet I have no permanent damage to arteries, according to the MRA scans. Was on fortnightly blood tests from April till last month as was tapering fortnightly. Now slowed down to monthly tapers. Main symptoms of the TAK flares(outwardly) were sudden big fevers, breathlessness, extreme fatigue (as opposed to the ongoing severe!), pulse 112-140 and irregular, and 'pneumonia' which wasn't but it looked the same. Hope this helps

[Hidden]

It's an interesting question. I *vaguely* had an impression that these conditions were somewhat "different" when I last looked. Having said that, I do get to read that different people have different symptoms and imaging results even within GCA or within Takayasu ? (Takayasu is incidentally, a Japanese dr's name who "discovered" this condition? ). Dividing simplistically, if you are younger (10-30 please not to quote me on this) you got Takayasu, if you are older (middle age?) then what you got is prob. GCA (whilst GCA is considered as over 50 condition, under 50 is also affected in GCA to confuse the matter): I am not sure how would this simplistic division actually "work"? There must be more than this "age thing" going on, I am assuming. Slightly, "chaotic situation" is happening as vasculitis is still a rare condition? To complicate the situation, lots of symptoms in different V may be very similar due to V being autoimmune... I would be interested to know myself. How old are you, Caro12line? It's a very valid question you ask. Thank you for sharing.

[orsen-trapp in reply to Hidden]

TAK - Also known as 'pulseless disease' but as far as I know they rarely had trouble finding a pulse with me. And supposedly goes with high blood pressure but when flaring mine was on the floor - and it's only normal now I'm on Pred (which raises blood pressure)! Which at least makes it easier for everyone to take bloods

[Caro12line]

Thanks orsen-trapp and Ashfordgreen. I am 58 so that makes me an "oldie" and in the GCA category I suppose. I have never had fever or trouble with finding pulse. But I do have high BP (on Amlodipine) and have rapid heartbeat and skips ( have had 24 hour ECG monitor) my GP puts all this down to the Pred. I will ask the question to my Rhuemy when I next see him in October. And that is another of my concerns...suspected GCA in Jan this year and put in high dose Pred immediately, TAB negative, other tests inconclusive, thought not to be GCA Tapered down to 10mg and flared. Had PETCT scan and confirmed GCA and Pred up to 40mg in May. Since then I have been left to taper on my own with GP to sort out the many side effects. So it does worry me that my GCA/Aorta are not being monitored.

[BronteM]

I've also been given the 'above and below 50' explanation so now just tend to think in terms of large vessel Vasculitis. However, I've never had headaches, but do have high blood pressure and no pulses in my arms, and the clinic now refer to me as having Takayasus. Unlike orsen-trapp I do have permanent scarring in my main upper body arteries, and the immunosuppressants have only improved the blood flow by about 15/20%. As the initial tests showed 90%+ blockages in my arms, that still means pretty severe problems! It also means that the scarring must have occurred over a fairly long time, so I've had the Takayasus rumbling along at a low level for a long time - probably since my late forties. The scans also show that I've grown a network of capillaries round the blockages, and this must have taken a long time too. This could be the case for a lot of other people, and makes the 50+ cut off a bit pointless.

[Hidden]

Good question Caro12line!

They are both a form of large vessel Vasculitis and can affect any large artery in the body.

I think that some Dr's think they are a continuum, usually but not exclusively TAK is a disease of young females ( up to age 40 ) and GCA manifests itself over 50. Goodness knows what happens in the decade in between but it is very hard to get a GCA diagnosis under 50!

You can get GCA exclusively in the aorta but you can't take a biopsy from it to look for giant cells, I think it depends what Dr you see whether it is named TAK or GCA.

You can get GCA by itself without PMR symptoms and vice versa.

GCA shows a prediliction for the temporal artery which can be biopsied safely to look for Giant cells.

TAK is known as pulse less disease but that is a late phase where the damage to the artery has been done and it's occluded. It's usually peripheral pulses that are absent ie radial but as its a long process collateral circulation is in place to maintain the blood supply.

I think common symptoms between the 2 are fever, fatigue, malaise, weight loss and joint pain. Most people get an increase in inflamatory markers ( ESR and CRP ) but not every patient.

The main stay of treatment for both is Prednisolone.

That is my simple understanding of both diseases, no doubt someone more technical can add to this!

[PMRpro]

As I understand it they are both vasculitides and age is the criterion for which name you are told - if you are under 50 it "can't" be GCA so you are told it is Takayasu's. That is a sweeping generalisation but you would not believe the number of doctors who truly are convinced that at 49 you can't have GCA but once you are past your 50th birthday you can. We have plenty of people on the dedicated forums who have classic GCA symptoms but are told it can't be since they are "too young". Two years ago a 37-year old man in South Wales died of a stroke. At autopsy the pathologist found he had had undiagnosed GCA. Pathologists know it ALL but it is too late!

PMR and GCA are also both vasculitides and one in 6 patients with PMR will go on to show symptoms of GCA - but that may be because PMR symptoms are also found in patients with GCA where the PMR is a symptoms of underlying GCA. However, they don't always appear together. Patients with PMR may have ONLY PMR and people with GCA may only show signs and symptoms of GCA. If you present with GCA symptoms you may never show PMR symptoms - it all depends on which arteries are affected.

And while I'm at it - do challenge you GP about the cardiac symptoms you have. I have atrial fibrillation which manifested like that and was definitely NOT pred, I wasn't on it. The cardiologist is of the opinion it is due to the underlying autoimmune disorder that is the root cause of both GCA and PMR (I have PMR symptoms but it is almost certainly due to GCA of larger arteries in the thorax but that is by the by). In the past they couldn't do the imaging - and they are still sticking by the story from 40 years ago. Much to the charities' annoyance. We see the patients who are left in limbo because they are "too young" and "atypical". For many a dose of pred achieves a minor miracle - which does suggest that the other suggestions of "fibromyalgia", "depression", "your age" and so on were a load of mdooly...

[Caro12line]

Thank you all so much for your replies. It is a very muddy area! Which brings me on to a second question that PMRpro might be able to answer. As I have GCA found in the Aorta should I be seeing a Cardiologist or a Vasular Consultant rather than Rhuematology? I briefly suggested this to my GP because of the abnormal heart readings and she said you are already seeing a Consultant. Which isn't really an answer but think she may have been reluctant to refer elsewhere as my Rhuemy is Prof BD. But I can't get to see him!

[PMRpro in reply to Caro12line]

I doubt it really - rheumies are a bit protective and will claim they specialise in vasculitis too. I think your GP is a bit naive - I could be seeing a consultant for a gynae problem but they wouldn't be much use for vasculitis would they!

However - if you have confirmed GCA then you should be under the care of a rheumy and not being left to the devices of a GP for tapering. Even if it isn't Prof BD (in sunny Essex I assume you mean?) then it should really be his team. Though was it them who decided it WASN'T GCA because of a negative TAB? Naughty naughty if so - at least half of TAB's are negative even though the clinical decision is one of GCA. Negative TAB does not rule out GCA, a positive one, however, is 100% sure.

However - monitoring of the aorta is more a long term thing. Ideally you would have an echocardiogram every couple of years - the monitoring is to look for an aneurysm developing in the thoracic aorta. You can also ask your GP to get you added to the AAA (abdominal aortic aneurysm) screening that is available for all men over 65 and other appropriate people which you are if the GCA was also seen in the abdominal aorta..

nhs.uk/Conditions/abdominal...

I suspect though that with having aortic GCA it wouldn't be just a single visit as described in the blurb there - the risk of one developing is down the line, as much as 10 years into the future. But you could discuss it with them.

What did your 24 hr Holter ECG show? Anything or nothing? I complained of the palpitations and stuff and found the GP quite dismissive - but he did say that if it was too bad at any time to dial 999. I thought that was a bit OTT but on reflection, no not really. The fairly certain way of identifying what it is is an ECG at the time - and paramedics carry the gear. Get them there when it is happening and they will find something - it never happens to order when the ECG appointment is booked!

And out of interest - how have you tapered?

[Caro12line in reply to PMRpro]

Thanks PMRPro.. I knew you would have the answers! Yes it was the good Prof in sunny Essex who said of my TAB result. "clearly negative. So we don't think it is GCA, which is good news, you don't want that." Then to his intern, "Taper her off the Pred and give her Pregabalin for the Neuropathic pain". It wasn't until the Pregabalin had completely Zombified me and the pain continued that his intern did an Ultra Scan in my armpits and called him him to see results and they sent me for a PETCT. All this took 4 months. Back up to 30mg of Pred and taper off the Pregabalin (that in itself is no fun!). That was back in May and I haven't been seen since, except by my GP (3 of them, it's a big practice)

The GP said, (in a dismissive way) the ECG came back ok. But same as you if the "fluttering and tightness" get bad, go to A&E

I will ask about the AAA, thanks. And when I go back to see the good Prof or his Intern in October I will ask about a Cardiovascular referal, so I have more idea of what is going on. What part of the Aorta is affected? Does it spread? How bad is it? What are my chances of heart attack, Stroke etc? And the one question they probably don't know is how do I tell if I am flaring or is it Pred side effects or withdrawal?

I Tapered to 10mg at 1 mg a month fine. Then tried the same with 9mg. The drop was too much. No pain but I could not function, manic episodes followed by uncontrollable sobbing. My husband took me to the GP who said my CRP was raised (21) and go back to 10mg, things settled down again one week later CRP 13 so GP said give it another week at 10mg then try a slower taper. So now I am trying the "tortoise not Hare" method. 11 days in and all ok, although I have no idea what my CRP is doing

Thank you for your help and advice

[PMRpro in reply to Caro12line]

I know PBD doesn't agree with the other expert I'm quite keen on but there is a reduction plan for GCA which spends a month at least at each reduction dose down to 20mg. Having started at 60mg. Then he reduces at the same rate as his recommendation for PMR - having got to 10mg he keeps his patients there for a year. What makes her think that if you couldn't reduce last week from 10 to 9 without the inflammation markers rising that you will be able to next week? So I hope she is willing to do CRP tests every few weeks.

If the dose is above what you need to manage the symptoms you will be able to reduce OK and you just need smallish steps to avoid the shock of the change triggering steroid withdrawal discomfort. Once you reach the dose you NEED, it doesn't matter how slowly you reduce, the symptoms will flare.

You have done amazingly well to get down to 10mg in 4 months - most people I know are above 20mg for over 6 months. But why oh why is there this desperation to add in other drugs - some of which are worse than pred.

Good luck!

[Hidden in reply to Caro12line]

No!

Vascular surgeons aren't great at medical management and cardiologists aren't the experts in auto immune disease although it may be an idea to see one alongside the Rheumy.

If you have concerns about your care you could always ask for a second opinion, Prof Justin Mason at the Hammersmith in London is a large vessel Vasculitis expert ( I am sure that BronteM saw him at one point). He is very personable as well, which always helps!

[orsen-trapp]

They spent a while as I languished in A & E arguing whether I was cardiology or rheumatology! The rheumies won. My rapid pulse lasted for 6 months, along with lots of missed beats, etc., until I was put on Pred ... at which point it became normal, apart from the jumpiness. So, Pred was definitely an improvement in that department. However, I'm the only one I've come across here or on facebook for whom that is the case! My TAK is just the ascending aorta, as far as anyone knows.

[BronteM]

I should ask to see a cardiologist...it may be a completely different problem to the Vasculitis. My GP sent me in for 24 hour monitoring after an unusual ECG. It turned out that I have a rather exotic form of atrial fibrillation that Papworth got quite interested in! It's nothing to do with my Vasculitis as far as anyone can find out, and probably hereditary. But part of my Takayasus is a very tiresome back ache at bra strap level which was quite frightening until I started getting proper heart checks and learning how and when it appeared, and how to deal with it.

[orsen-trapp in reply to BronteM]

Last sentence, Bronte - how do you deal with it? I've not had it for a while but if it reappears ... and what is it?

[BronteM]

Not sure really...but I met another Takayasus patient yesterday who has the same problem. She is also in the 50+ bracket and has been swivelling between GCA and TAK.

I now use mine as a warning sign; it's time to sit down and take it easy. It seems to happen when I've been doing anything that involves standing or walking for more than about 20/25 minutes, and that can range from pottering with my elderly Labrador to a baking session or wandering round a museum. The worst I've had recently was at my niece's wedding which was very boring.

I think I've convinced myself that it's lack of oxygen in my narrowed and rather distorted aorta, and a bit like the claudication in my arms. I keep mentioning it, but don't seem to get much of an answer about what causes it....just the advice 'not too worry'!

At my last appointment the cardiologist said that he was 100% sure that I wasn't going to drop dead as I walked out - my statistics training kicked in and I pointed out that it would be very inconvenient if I did! But then he added that the most likely time for the fibrillation to kill me was in my thirties, and I'd obviously survived that....