Primary aortitis

Hello everyone, I was diagnosed with aortitis 1 year ago. It is only now, that I have made a decision to share my experience. It has been difficult to find and share advice about my condition, because it is rare. My type of aortitis is with no known cause, I began to feel abnormally fatigued, with drenching night sweats, feverish episodes an strange chest pain. My gp took some blood tests and these showed abnormal crp levels of 210 (normal value is less than 5), abnormal liver function tests and mild anaemia. To cut a long story short, I spent time in hospital, having a lot of scans, blood tests, iv antibiotics for possible infection etc. 5 weeks later, I had a PET scan which showed an inflammed aorta. That was December last year. I have been on prednisolone, which I am currently tapering down, because I am now taking mycophenolate. My crp levels have come down dramatically but not normal yet. They are fluctuating between 15-19. Noone can tell me why they havnt returned to normal. I am better as far as my blood tests go, but I still feel fatigued, I am overweight as a result of taking steroids and I feel generally weakened. I don't think that I will ever be as robust as I was previously, but at least I am still alive. I have blood monitering done every month and I see a rheumatology nurse specialist every 2 months. I would be interested to hear from anyone who has this condition, and how they have coped with it.

34 Replies

  • Hi there,

    I think that the reason your crp levels are slightly raised could be solely down to being on the prednisolone... even on a lower dose whilst tapering. Taking any amount can apparently raise crp results.

    Just a thought, all the best,

    Will 😊

  • That is news to me - it is something that I have never come across and I and all my mates on my home forum (PMR?GCA) are on pred and only pred for a long time. It is very unusual for someone to have a raised CRP while on an adequate dose of pred and when it is almost always because of a chest/respiratory tract infection. The CRP is a typical finding in both PMR and GCA and falls dramatically as soon as pred is started, often being used to monitor disease progress. The aim is to get it into the normal range and rises tend to indicate a flare of inflammation.

  • Hi PMRpro, I assumed that that was the usual procedure for my condition, but of course, we all have immune related conditions. When my consultant rang me, after 4 months of treatment with prednisolone, he said that he wanted to add-in the mycophenolate, as, and I quote, "your crp is not coming down, as quickly as we would like". Up to now, I havnt really had any infections per se, apart from a minor outbreak of shingles. I also have chronic oral thrush, which I cannot get rid of....ive trolled the internet, and nothing seems to work. 😒

  • Hi Will, an thanks for that, it sounds interesting. My specialist nurse coudnt tell me the reason, but suggested that my CRP levels may always have been in the range of 15-19. I must say tho, that when I began prednisolone at 30mg a day, my CRP dropped dramatically. Of course, we are all aware of the vagaries of prednisolone, including the long term effects, even a year after stopping it.

  • I was diagnosed with PMR and temporal arteritis four years ago. All was fine as long as I was on Prednisolone and/or Methotrexate but as soon as I came off them my inflammation levels shot up again. This summer I had a PET scan which showed inflammation in the aorta, the clavicle aortas and the other major blood vessel joining them - forgotten its name and I may have got the other ones wrong, but basically most, if not all of the major arteries in my chest. Now on Prednisolone and Metoject and tapering and inflammation levels are normal. Unlike you, I had NO symptoms and, in fact, felt, not just OK but positively well! I thought the rheumy was fussing when he kept suggesting a PET/CT scan. How wrong can you be? Still, it is apparently a very rare condition so interested to hear of someone else who has it.

  • Hi Cornishjan, you have certainly had a bad time of so sorry..I did read on the internet, that in some cases, people display no symptoms whatsoever, and the condition deteriorates until some 'catastrophy' occurs and its diagnosed in hospital. I'm pleased you have a dedicated rheumy and are stable just now. I've often wondered how long it takes to reach full remission, and not have to take these awful medications....yes, they save lifes in one respect, but the worry is the constant state of immunosuppression. Its a double-edged sword.

  • People with RA and the like take these medications for the rest of their lives because they rarely go into remission - the immunosuppression is to allow you a life and, psychologically and emotionally at least, it does help in the long run to become friends with your medications rather than regard them as "awful". I had my vasculitis for 5 years without steroids and I have no desire to go back there and I have had a few doozy side effects in the past! Aside from anything else the inflammation itself causes long term damage if left unmanaged, but a life of being unwell and in pain is no walk in the park.

  • I understand that, and you're right, of course. My rheum team did put a lot of emphasis on 'getting off the steroids' and I've thought of them as awful ever since. From what I understand, the lower the dose of pred, the less likelihood there is of damaging side effects, and yes, I agree, that the inflammation causes more damage....its a calculated risk.

  • Suzycat: What is the relationship of your particular aortitis to Takayashu's arteritis? Tocilizumab has been approved for the NHS to use in Takayashu's (though not in GCA which is to all intents and purposes identical except for the age at which it is diagnosed).

    I found this interesting;

    I think you were very lucky to have a rheumy who WANTED a PET/CT scan - a lot of us on my home forum would have loved to have one done.

  • Hi, PMRpro, my consultant referred to my condition initially, as Large Vessel Vasculitis, then at a later stage, Aortitis, he hasn't classified it as anything else, although I have read about Takayasu"s and my symptoms fit. I have a young rheumatologist, but he wasn't the one who persevered with my tests, I was initially under the care of an Infectious disease consultant, I'm assuming, because of my fevers etc. At the end of the 5th week of testing, he was still no clearer about my diagnosis. That was when he referred me for a PET scan and yes. I am lucky, and I'm very grateful for that. I have read some cases on here, that makes me want to cringe at the medics, for their blase attitude.

  • oh, thank you for the link, I will read it 😊

  • I also have aortitis and receive my treatment at the Freeman in Newcastle. I was very lucky as I was being assessed for various types of cancer as I had severe abdominal pain, massive weight loss etc. A ct showed aortitis and I was prescribed steroids and MML BY A rhumatologist at my local hospital. That was four years ago. I asked to be referred to the Freeman and now see a rhumatologist every three months. I have recently had my meds changed to Azathioprine. I am also on 7mg preds. I am by no means 'better' but lead an active life. Good luck!

  • Hi Annsouthren, I had my PET scan at the Freeman, now there's a coincidence! I wonder If we have the same rheumatologist?? Have you been advised to taper you're prednisolone? My specialist nurse strongly advised it, because of long-term adverse side effects, like for example, osteoporosis and cataracts oh, and diabetes. I am tapering very very slowly and down to 2mg just now. How long did it take you to feel more energised? Have you had any flare - ups?

  • Hi What a coincidence! For three years I saw Dr Peterson, she left and I now see Dr Vila. Everyone in the Rhumatology are great. As for meds, once I get to 7mg I get severe joint pain so the doc wants me to stay there at the moment. I am determined to get off steroids but it may never be possible. As for health I can't really tell! I'm very tired but have never had the severe pain I had pre diagnosis. I think my problems are steroid related and hopefully, with reduction they will decline. I asked my rhumatologist if it was the same as TAK and she said because of my age at the time , I was 63 she wouldn't consider it. I hope your treatment at the Freeman is as good as I get as I've been amazed with the standard of care I've had.

    Ps Suzycat I live in Durham

  • I don't receive my treatment at the Freeman, I only had my PET scan there....Dr Goff is my rheumatology consultant and like you, I feel that I receive a high level of care. I think he is based at Wansbeck hospital, but probably covers the whole of Northumberland. I see that a lot of people have severe joint pain on here and I feel for you all, I really do. For all I am now down to 2mg of pred, I am unsure as to wether I will feel ill again when I drop the dose again..its not easy. I was 56 when I took ill and I live in Morpeth. I am lucky enough to live near the new Northumbria Urgent Care hospital, which is where I was admitted initially. Top notch staff and state of the art equipment.

  • I used to live in Durham, Fram first and then Pity Me.

  • I've visited Durham, lovely place. Don't think I've been to Pity Me. I'm thinking its a small rural area?

  • Suzycat:

    It's right on the edge of Durham, Pity Me, Framwellgate Moor and Durham city are all joined up - though looking out the front windows of the house we could see fields. It was a 10 min walk to the Arnison Centre and S'burys as our corner shop!

  • ahhh...lovely!

  • I was Deputy Head at the special school at Aykley Head s!!

  • I've been on pred at a higher dose than you for over 7 years - no change in bone density, no cataracts (I wish - would love to be able not to have to wear specs!) and no diabetes. It doesn't have to happen as a lot of us would tell you! If you are managing fine on a lower dose that is great - but don't panic if you can't.

  • That's good to hear, its bad enough having the illness. Saying that, my consultant says that all meds have side effects, so basically we put up or shut up. Haha...i have my blood taken to check bone density and I take Evacal calcium and vit d chewable tablets. No problems as yet! My consultant wanted the mycophenolate to replace the prednisolone. I'm now into my 9th month of treatment. Its reassuring that none of you have had significant problems with prednisolone, my main problem has been 4stone weight gain...oh the joys!....

  • It IS possible to lose weight while still on pred - I managed to lose 36lbs starting at 15mg. I cut carbs drastically - and it is the only way for me, if I eat more carbs than I know is good for me, no weight loss, too much and I gain again! Others on the PMRGCA forum also avoided weight gain or lost weight the same way.

    There is a thread on the LupusUK forum on here where a few ladies have reported on their success in losing weight while on pred using the Slimming World diet (some of them a LOT) - and my daughter has also lost about 8kg of her pred weight using SW.

    Blood tests for bone density? That's a new one on me - it's a dexascan you need to know the state of your bones. As far as I know they wouldn't use it if you could just do blood tests - they are to monitor the status of treating a problem.

  • blood tests must have been for vit d levels, I havnt had a bone scan. Should I be having a bone scan at this stage? I love carbs too...oh dear 😞

  • Ideally a dexascan should be done as early as possible when taking steroids and then repeated every 2-3 years, different places have different regulations.

    Pred changes the way our body processes carbs - and that results in the deposition of fat stores in the usual places: round your middle, around your neck and on your face.

    I know - UK/US food tends to be very carby doesn't it! I gave up a lot of carbs years ago when I realised I had a wheat allergy (PMR gets the blame) so already didn't eat processed carbs. They are a treat now - and when I see what the scales do either way it does help!

  • Hi I have a bone scan each year and so far my PET scan has been repeated annually. It was my Rheumatologist in Durham that suggested I transfer to the Freeman. When I transferred a found their specialism far more specific-might be worth considering.

    I too have gained weight, 3 stones and it may just be my New Years resolution JOIN SLIMMING WORLD!!

  • Lucky you - I had bad experiences with the 2 Durham rheumies I saw. One was a colleague of my husband so I was a bit surprised!

  • Well, I've just got a brochure for Wiltshire farm foods, they do reduced calorie meals/smaller portions etc...methinks I'll give them a try soon...i'll ask my specialist nurse about having a bone scan. I asked my consultant a while ago about repeating my PET scan, he said at the time, that he didn't want me to be exposed to too much radioactivity.

  • PET scans are relatively high dose - a dexascan is low, less than 2 days exposure to natural background radiation and about 2/3 of what you get from an ordinary x-ray - and less than a third of a plane trip to the USA!

  • Anyone with aortitis should probably have a newly discovered condition called "IgG4-related disease" (IgG4-RD) excluded -

    IgG4-RD is not yet well known by most doctors, including rheumatologists. It can affect anywhere in the body, though in any individual it tends to affect either one area only or more commonly a few different areas. It typically responds well to steroids, but is prone to relapse -

    PET/CT scans are particularly useful for detecting IgG4-RD that involves the aorta -

  • Hi MacLondon, thanks for that...ive never heard of that one...I'll read your links an get back to you with a longer reply 😊

  • hello again Maclondon, I read with interest, your links about igG4, one of the links I couldn't open as my mobile phone browser doesn't support it. Now, this is interesting. My pet scan showed aortitis and my blood results at that time showed crp of 210, raised esr, abnormal liver function tests and slight anaemia. If my condition was caused by igG4, how would it show up on a pet scan and be easy to diagnose. I actually saw my results, and all I could see was the characteristic orange colour of my inflamed aorta. Does igG4 show up in blood tests? If this is the case, then my primary aortitis could in actual fact, be secondary...due to igG4. My crp levels responded dramatically to prednisolone and I was later prescribed mycophenolate, as my consultant thought that my levels weren't coming down fast enough. My CRP one year down the road, is between 13-19, and noone can tell me why, but there was a suggestion that my 'normal' crp might have been above the usual count of 0-5. I have an appointment with my consultant in March and I am going to ask him about it. I would welcome comments from you all please 😊

  • Hi PMR pro, this has clarified my understanding,now, of makes perfect sense. I'm going to ask my consultant about this. Thankyou 😊

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