14 years and oh dear, the journey is not over yet!
After 7 months off all meds, had a PET scan Xmas eve which showed GCA in my aorta from heart to abdomen. Back on Pred and Actemra. At least we know where it is now, as I’ve mostly had constitutional symptoms.
I have 2 questions: what us the usual monitoring for disease activity and aortic aneurysms? And, with aortic involvement is it sensible to be followed by someone with vascular expertise?
Discouraging to say the least..,
Sorry to hear that.. you can see related posts now you have asked, and other will be along with their experiences. Also see this from the FAQs -
Hi. Sorry to hear this. I'm not familiar with the effect of GCA on the aorta, but with that length of involvement you are correct in thinking you ought to have aortic specialist expertise. Please have a look at our charity's map of aortic specialist centres, here aorticdissectionawareness.o... Whilst that list says "aortic dissection" these centres are also very active in assessment and preventative work such as you will probably need.
In a rather odd manner (since the aorta has not until recently been treated as an organ in its own right), the aorta is not entirely the domain of vascular. At the heart, valve, and in the ascending aorta and arch it is generally dealt with by cardiothoracic surgeons, whereas the descending is dealt with by vascular specialists. The centres listed will have both and will work as MDTs, Multi-disciplinary-teams.
Very best wishes.
p.s. Searching on our charity's support forum (Facebook links are on that same website), we have a number of members with PMR / GCA.
Not much use to PMR2011 as they are in the USA,
thanks, as you say, for me, it will be monitoring for formation of aneurysms. I am in the states so I will start looking around for someone in vascular who has GCA experience. I’m also hoping to find information on how the giant cells affect the aortic lining and what kind of time frame the monitoring should cover.
A baseline is the basis for frequency - if there is nothing to see, you wouldn't be likely to need annual. Probably doesn't need GCA experience either - an aneurysm forming is an aneurysm forming, the underlying reason is immaterial.
Except aneurysms do grow at different rates and/or fail earlier depending on the underlying reason. For example, connective tissue disorders such Marfan and Ehlers Danlos result in earlier preventive surgery at lower diameters (or earlier dissection if the aneurysm has not been found incidentally). As well as known genetic causes like that, probably a third of dissections occur with a family history but no known gene mutation, i.e. it is genetic, but unidentified, so they are also generally treated earlier. There are also quite a few cases where dissection occurs without an aneurysm.
It's true no definitive cause has been found for aneurysms or dissection, but cell structure, inflammation, atherosclerosis are all in the mix.
GCA is a particular risk factor, and aortic centres will be aware of some of the applicable risks and differences to other aneurysmal presentations.
The subject is covered on page 66 of the 2024 European Guidelines, see academic.oup.com/ejcts/arti...
I also have LVV in my aorta and its branches. I have had one PET-CT scan at diagnosis, but the monitoring is by way of my CRP level. I realise this will not be possible while you're on Actemra.
I had my UK 'ration' of Tocilizumab and am now back on prednisolone, so we are able to continue to check my CRP, which remains low. I see my Rheumatologist in 6 weeks' time and intend to ask her the same question - how do we really know what's going on?!
Let us know how you are.
PMR: 2016
GCA-LVV: 2019
My rheumatologist's response to that one was 'We don't know', but there's a limit to how often you can have scans with high radiation, and that's the only real way to tell.
Not true - see my reply below. Thoracic aneurysm shows on echocardiographs and MRI - no radiation. There is an excellent AAA (abdominal aortic aneurysm) screening in place in the UK - ultrasound, no radiation. My husband was involved in its development - a vascular diagnostics specialist at the time.
Ultrasound is fantastic! It’s a great shame that not every rheumatology clinic is equipped. Expense is a major factor and training is lengthy as you will know.
It isn't as bad for a single structure - but what IS essential is doing it regularly. My colleagues used to say they could feel the difference after a 2 week holiday!! You have to keep your hand in - and that is one of the primary problems with GCA u/s, it isn't seen at clinic often enough.
Could I just chip in here to say that I was diagnosed with GCA four and a half years ago, ( I live in London) and after a week of daily massive antibiotic infusions and MRI, ultrasound and every other possible scan, it wasn’t until I was sent to the next door hospital for a PET scan that they identified the inflammation in left temporal area and main aorta as GCA. ( Still reducing, currently down to 3 and a half Pred, but last time I got to 2, back up to 60. I used to enjoy snakes and ladders…)
That is for the GCA, the u/s is not for GCA in this case, it is for the structure of the aorta. Different thing. GCA u/s only works in arteries where you can visualise AND exert pressure with the probe. It isn't looking for the inflammation. And PET-CT isn't an option for screening - too high radiation dose.
Worth adding that echo cannot see the whole of the aorta. It can see the heart, aortic valve, ascending aorta, only some of the arch, then not much until you get to the abdominal aorta (AAA territory). We have a fair number of members who have had dissections through only partial scanning of the aorta, and the dissection occurring where they weren't scanned. The aorta such a long vessel, with 4 different embryonic origins hence structure along its length, and can fail anywhere depending on underlying cause. The failure may also occur elsewhere than the obvious aneurysm - mine failed in the arch where it had not been growing, not the ascending, which had been. The rule is scan the whole aorta.
CT technology has over the years been reducing the amount of radiation, such that usually the risk of radiation is smaller than the risk of not scanning. The standard monitoring regime after an AD is, for example, 3 months, 6 months, 12 months, then annually. This is what I had after my AD, and have at 5 years gone to every 24 months. (I'm now 7 years after).
MRI is about equal to CT in terms of aortic imaging quality, and remains a good radiation-free option, especially as it (and CT) can see the whole aorta and use ECG gating to "freeze" the pumping motion to get a clearer image.
That's right - but these are things that are introduced after an aneurysm is established. Otherwise you would have to screen every GCA and LVV patient from start to finish which is probably totally impossible in most healthcare systems and certainly in the NHS at present.
Yes monitoring is difficult. I first had PMR in 2017 then LVV 2020. Had PET CT at diagnosis then again 2023. Second scan showed sub clavian artery inflammation had settled but ‘tramline’ inflammation in aorta. Better than in 2020 thankfully. I’ve been taking Sarilumab since one year Tox completed so markers suppressed. Also had Rituximab last year. Trying to keep B cells depleted currently and going by my constitutional symptoms.
I have had no satisfactory response to this question. The consultant I have now is happy to keep me onToc as I soon became unwell when it was stopped after over 2 years. The second ct pet scan showed activity in the same area as the scan at diagnosis but not quite to the same degree. Surely some sort of follow up should be performed???
Hard luck - the numbers of this group are increasing!!! LemonZest11 is in the same situation. Rugger has replied and SheffieldJane is another to add to the list. Interesting this has all come to light in the last couple of years. Makes you wonder how many of us with Long PMR actually have LVV. I had a PET-CT that didn't find anything but I was on 7mg pred at the time so possibly unreliable. I'm on pred and Actemra anyway - but that's while I am here in Italy, the UK might be another matter.
Was there any sign of distension of the aorta in the PET-CT? Aneurysms develop slowly and if one is identified it is a monitored to see how it develops. If it is static at under a given dimension, then nothing is likely to be undertaken since the options have considerable risks themselves and everything depends on the balance of risk. It will depend a lot on what country you are in as well.
In the UK, there is a general screening process for AAA - abdominal aortic aneurysm - but thoracic aneurysms are a different kettle of fish. And since you are in the USA - I have no doubt that that is in the hands of the vascular surgeons! Thoracic aneurysms might also come under the remit of the cardiologists though as it may require cardiac surgery.procedures.
Just looked it up - and Johns Hopkins says 6 monthly CT-angiograms which seems a bit steep in terms of radiation! I imagine that may be for one that they know exists and is above a given size. Diagnosis is often via cardiac MRI (no radiation - but if you have metal "bits" it isn't advisable)! I have regular echocardiography which also detects them - no radiation and metal isn't a problem. Even a plain x-ray will indicate the possible prescence and is what is recommended in the guidelines for GCA - but rarely carried out!!
Glad to hear that ultrasound has advanced enough to look for abdominal aneurysms. I’m assuming that’s fairly specialized. I thought to start making inquiries with vascular departments to see what they have to offer for GCA monitoring.
do you know of any good article articles that might speak to the course of GCA in the aorta and it’s potential effect on the vessel walls?
As ever thanks for your incredible contributions to this site.
That was years ago - at least 25!!! Not really - you just have to be trained to use the device and recognise the signs, technical staff do it in the UK and it doesn't take long. I did ask at one time and was told it was possible to be added to the screening at a different age and as a woman. That may have changed. Thoracic is more complicated though because of your ribs.
nhs.uk/conditions/abdominal...
I was talking with Prof Quick about it the other day - slow progression and usually after some years of inflammation was her assessment so no point panicking about it at the outset which was what underlay my query (a post). But yours has been around for a while.
I think you could start by asking your GP how you get referred for an echocardiogram after requesting a chest x-ray - the aneurysm would alter the appearance of the bit where the heart shows on it. But you must have offices that do that sort of thing - providing imaging services to doctor's offices? They sell everything in the USA don't they?
Hah, probably true.
The good news is the CT they did with the PET scan didn’t show any aneurysm (and no atherosclerosis!). So it’s really just monitoring moving forward in case there was some loss of wall integrity.
Re articles on GCA in the aorta, the European Guidelines on aortic disease cover what relatively little literature there is, page 66: academic.oup.com/ejcts/arti...
I’m a patient at Johns Hopkins and they don’t know anything… my rheumatologist even admitted it. So I’m on the of prednisone. He’s very lackadaisical in contacting me. When I met him at first he said he swore he would get back to me in three business days after sending messages through the portal , because of the attitude I had with the last rheumatologist. Well it’s been over a month for the second time.
He told me all he needed was blood work to tell me how to titrate. He ordered the bloodwork but asked me to send the results to him. Like wouldn’t he have results to the order he submitted?
At a recent support group where my retired, knowledgable rheumatologist was in attendance, I asked him by which method and how frequently did he advise monitoring a 4cm ascending aorta aneurysm and he replied, “Echocardiogram-yearly.” Yes, you should be managed by a vascular specialist or at least a rheumatologist who has an interest and who will listen, who will enter into discussion with you and who will involve team members if necessary.
Morning, I was very interested in your post. I was diagnosed with PMR 7 years ago. Started having bowel problems in the summer. My GP referred me to the hospital. Had CT scan which showed bowel, stomach and aorta inflamed. Referred to rheumatologist , had PET scan. Showed aorta, subclavian, carotid and femoral arteries all inflamed. I am just starting on methotrexate as well as 20mg prednisolone. Like you ask, how worried should I be ?
Oh dear, I am sorry to hear that.I can completely relate to your concerns. I was diagnosed with suspected LVV GCA in May 24, confirmed with PET CT in October. Non cranial. Aortic and subclavian arteries only.
I was gutted and terrified. My dad underwent life saving surgery 15 years ago for an aortic dissection so I was scared.
But my consultant (we come under nephrology at Aberdeen) explained it to me like this.
I have no signs of aneurysms and if there were any signs of AD I would have been admitted. Their plan to monitor is yearly PET CT. They say aneurysms develop slowly. I also have had slight raised BP for around 10 years always controlled by "diet and exercise". Now it's treated "to prevent the risk of mechanical failure".
I feel reassured. Not complacent though as I know these are my biggest risks - and fears. But I guess prevention through controlling the inflammation and controlling the BP feels like a fair plan.
Though I agree with others that having the option for a non radiation scan more regular might reassure / inform us more.
I am now down to 8mg pred and 2mg mycophenalate mofotil daily. Time will tell.
oh dear that’s pretty disappointing! But thank goodness they caught it and you are now receiving treatment to avoid worse. I hope you’re taking great care of yourself.
I was diagnosed with PMR, GCA by Temporal Artery biopsy, and LVV 3 1/2 years ago. This included Aortitis. I was on Prednisolone starting at 60mg but have just stopped taking it having reduced slowly. Had a couple of flares on the way down. I started Methotrexate 3 years ago and am still on this drug. I am closely monitored at the Vasculitis clinic in Cambridge. Scans are MRA so no radiation. My Aorta has much improved and no sign of Aneurysm. The consultant tells me that people do not tend to get them. I assume that I will have scans every so often. Had to wait 6 months to get the last one. I am really inpressed with all the care I have had.
I am now feeling well and getting on with life!
Very heartening to hear. I was first diagnosed with GCA in 2019. Though we didn’t know where it lay as I was on low-dose prednisone for PMR and my symptoms were constitutional. Thought I was done with it when I came off meds this past April, but looks like I was wrong. What I don’t know is if it’s been in my aorta all along.
how often do you have your MRAs?
GCA and Polymyalgia 
LVV Update
GCA and Vision update 
PMR/GCA monitoring and bone supplements 
