Incidence of Large Vessel Vasculitis in late diagnosis of PMR

[123-go]

When I read members’ experiences of prompt PMR diagnoses I’m truly pleased for them but I have to admit to feeling a degree of envy. My diagnosis arrived ‘only’ five months after onset of symptoms; I say ‘only’ because I know that for others the wait was far, far longer: PMRpro, for example, was without treatment for five years! This is hard for me to even contemplate!

My own relatively short undiagnosed journey to the eventual prednisolone and blessed relief consisted of increasing symptoms and approximately two-weekly blood tests where after each one the GP read my results and apologised for being unable to suggest a cause of my increasing pain that was gradually affecting every part of my body from head to toe and fingers: inability to lift hand to mouth, daily weight loss, non-existent appetite, weakness from head to toe, deathly pallor and eventual CRP level of 115 -see above (ideally should be below five).

It was after being told for the eighth time, “We are still trying to find out what is wrong” that I crumpled and told the GP that I couldn’t go on for much longer. I shuffled out of the door, hunched over and in extreme pain out after having to be hauled up from the chair because there wasn’t a hope in h*!! that I could manage to independently stand from seated. Within a week I was referred to Rheumatology and thankfully found myself in the care of a renowned specialist who had spent most of his working life researching PMR and GCA/LVV.

Lately, I have come to believe there must be a link between late PMR diagnosis and lack of treatment where inflammation in the body is allowed to build up to such high levels and lead to Large Vessel Vasculitis and aortic aneurysm and/or other conditions.

My GP could see my rapid physical deterioration and worrying blood test results.

Why wasn’t inflammatory disease investigated?

Would my aneurysm have been prevented if I’d been diagnosed sooner?

I don’t think I need to ask if my GP (kind though she was) was lacking in knowledge/awareness of PMR.

I would be interested to hear from others who have had a similar experience of LVV after delayed treatment for their PMR.

Thank you for reading this 😘.

[PMRpro Ambassador]

Don't know if I have LVV as the cause of my PMR - very likely as I did have a short period of very GCA-ish symptoms with jaw and thigh claudication and a few other things but they resolved on their own, something that has been noted as occasionally possible, the autoimmune bit just aborts and the GCA disappears.

To my knowledge I don't have an aneurysm - certainly don't have a thoracic one as that would almost certainly have been found on the echocardiograms I have had.

[123-go in reply to PMRpro]

Thanks for replying, PMRpro. I may have misled you or didn’t make myself clear ☹️. I’m questioning the possibility of LVV as a progression of undiagnosed, unmedicated PMR.

[PMRpro Ambassador in reply to 123-go]

Yes - but it was well into PMR that my GCA symptoms appeared, I think I was very lucky.

[123-go in reply to PMRpro]

You were indeed. 💐

[123-go in reply to PMRpro]

Thought it’s worth mentioning that I had no symptoms of LVV/aneurysm. I don’t know exactly what alerted my rheumy to the possibility but he got the MDT involved: he obviously had his suspicions and those were confirmed by means of ultrasound on the very day that he told me of his imminent retirement. It was all a lot to take in at the time and afterwards there was a period of inconsistency when I was seen by several different locum rheumatologists until a permanent replacement was found who had been trained in u/s by my previous knowledgable consultant. I also consider myself fortunate 🙂.

[Omakaat]

Hello 123 go! Yes I thought the very same, at least when first diagnosed. Much the same story as yours, a GP who referred me to a psychologist for extreme fatigue and depression and gave me a reprimand for being lazy after my retirement. Get up, do something! Be more active or you will be so stiff! Well, she was right about the stiffness. It took six months to get a correct diagnosis. And I also more or less blamed the postponement of treatment on the long term full blown PMR and subsequent GCA (confirmed by PETCT scan).

But later on, I was reminded by a friend that my eyesight had been failing me before the onset of PMR symptoms. Because my eyesight always returned to normal after 20 minutes or so, I never gave it a second thought.

The first time it happened I was staying with my friend for a holiday and mentioned it to her in passing, I blamed it on being exhausted after a long hike. It did occur infrequently in the following months, but always connected to fatigue. Stupidly I was never worried.

So, it turns out it was more likely the other way round for me, first GCA and subsequently PMR.

I was so, so very lucky not to have lost my eyesight. My Gp not getting the PMR was no joy, but it did not lead, at least I don’t think so, to GCA. I did speak to the GP after th diagnosis by a rheumatologist and she apologized, having never even asked for a bloodtest. It turned out she did know about PMR and GCA but never asked about any other symptoms but extreme stiffness and pain and being so tired and depressed. And I always worry about taking up too much valuable time by complaining about things that come and go all by themselves, and do not seem to be very important. I always cycled to the doctor’s practice, went on with daily life so there could not be anything seriously wrong with me, or so I thought.

Live and learn, still figuring out my “user’s manual” etc. This forum definitely helps, when it comes to PMR/ GCA.

[123-go in reply to Omakaat]

Yours is certainly something of a horror story, Omakaat! At the very least I had the blood tests even though the results didn’t ring alarm bells with the GP as to the possibility of PMR. Stories like ours illustrate the importance of raising awareness of these autoimmune diseases particularly in primary care.

I’m not quite sure of the accuracy of the these figures but I believe that around 10% of PMR patients go on to develop GCA and 50% of patients with GCA get PMR.

As for the GP not requesting blood tests all I can say is, “Saying sorry butters no parsnips!” 😡

Questioning professionals can be alien to those of us who were previously healthy, active, and rarely needed a GP but many of us have found that we must be our own advocates. We have every right to respectfully ask questions and to expect respect in return. Here is a useful acronym: DBFO…..Don’t be fobbed off!😉

I am also constantly learning thanks to this forum and that is empowering. I wish you well as you journey forwards.

[DorsetLady PMRGCAuk volunteer in reply to 123-go]

Crossover of PMR/GCA

PMR is observed in 40-60% of patients of GCA patients at diagnosis.

Between 16-21% of PMR diagnosed patients may go on to develop GCA throughout their PMR life.

[123-go in reply to DorsetLady]

Thank you for the correction 🙂.

[DorsetLady PMRGCAuk volunteer in reply to 123-go]

It does seems to vary a bit depending on what study you read - for example this update from ARC [American College of Rheumatology] last year

At some point, 5 – 15% of patients with PMR will have a diagnosis of GCA. About 50 percent of patients with GCA have symptoms of PMR. The two conditions may occur at the same time or on their own. It also affects the same types of patients as does PMR. It occurs only in adults, usually over age 50, in women more than men, and in whites more than non-whites

[DorsetLady PMRGCAuk volunteer in reply to Omakaat]

See my reply to 123-go re overlapping of PMR/GCA - so you probably had PMR all along but didn’t realise it

healthunlocked.com/pmrgcauk...

[random901]

Morning, 123-go! When I was eventually diagnosed with GCAPMR October 2023, my rheumy said "Your bloods haven't been normal for the past 10 years." She also said that I had had these conditions "for some time." Oh yes indeed. 2013: hemorrhage in one eye, blind in it for 2 weeks. June 2022: thoracic aneurism resulting in major surgery and reinforced aorta. Persistent fatigue, pain and stiffness for 10+ years, diagnosed as fibromyalgia. After the heart surgery I was in severe pain for months. Yet no-one at any time investigated the possibility of an underlying cause. It was only after a GP visit because of sharp earache September2023 that I was immediately put on pred pending investigation for GCA. Delayed diagnosis could have proved dangerous, possibly fatal. Now making a very slow recovery (I hope!)

[Judyliz in reply to random901]

Yours is a truly terrible story. We read some shocking histories on here and this really is a tale of being let down and failed. I am very sad on your behalf and wish you all the best for the future.

[random901 in reply to Judyliz]

Thanks, Judyliz. All the best to you, too!

[Sophiestree in reply to random901]

no words, just WOW

[123-go in reply to random901]

How badly you’ve been let down!

Thank you for telling your story which made me want to weep for you while at the same time feeling angry that lack of investigations and inquisitivity have led to the position you are in today. Thank goodness your GP was on the ball.

Please keep us informed of your progress: I would like to offer a gentle reminder that you have a voice and the right to speak out if you aren’t sure that things are moving in the right direction. I wish you positive outcomes from now on.❤️

[random901 in reply to 123-go]

Thanks, 123-go. And all the best regarding your progress,too!

[123-go in reply to random901]

😍

[Numptybrain]

I think it’s the same for any autoimmune, it’s taken me over a year even though the respiratory team have been saying autoimmune all along the rheumatologist wouldn’t get onboard because bloods said no. He’s now had to bite his tongue and work with respiratory because the steroids he put me on he thought wouldn’t do a thing , have actually worked and are stopping the inflammation so hey ho experts can be wrong.

Take care

Wendy x

[123-go in reply to Numptybrain]

Hello Numptybrain (should be ‘Numptybrainsometimes’ like a few of us here😘).

Well, we know that often patients’ blood results are shown within the normal range even though they have inflammation in the body. Let’s hope that the rheumy has been educated enough to accept that anomalies can and do occur. Keep well and keep taking the little miracle pills x.

[Numptybrain in reply to 123-go]

I know originally inflammation was showing but now due to prednisone it’s not so that’s saying something. I know a rheumatologist years ago once suspected ankylosing spondylosis but then dismissed me and diagnosed fibromyalgia, I now think he was wrong as my back pain is horrendous when I go to bed. I actually thought after my spinal surgery, it would be a miracle cure as I had to use a wheelchair for outdoors prior but now it’s just the same only night pain is really bad something I haven’t had chance to discuss with rheumatologist as main concern has been heart and lungs.

[PMRpro Ambassador in reply to Numptybrain]

There is one heck of a difference between AS and fibro!!!! I would have thought the imaging they will have done for spinal surgery would have identified AS - but maybe it can be overlooked if you aren't looking for it.

[Numptybrain in reply to PMRpro]

As you can imagine my daughters are fuming from all of this as for years they have seen me struggling with my mobility with no answers and basically left.

[123-go in reply to Numptybrain]

At least the steroids are doing their job!

I can understand that some things - sometimes- have to take precedence over others but the ‘whole’ person should be treated (my non-medical opinion only) and consideration given to all symptoms; they could be connected. Do try to get a word in about your horrendous back pain. We go to bed to rest in order to face the new day and if your pain prevents you from achieving that the cause should be investigated. Perhaps one of your daughters would accompany you at your next appointment to support you. I’m assuming you are being reviewed on a regular basis.💐

[Numptybrain in reply to 123-go]

Yes my daughter is coming to respiratory on 8/5 they are more understanding and willing to help, it was unfortunately the rheumatologist that at first wouldn’t listen to the respiratory team.

Thanks

Wendy x

[Zebedee44]

Difficulty in obtaining a diagnosis is the theme of the study Lucas Coutin is conducting with PMRGCAUk to present in Leeds. I have submitted a short video of my own experience in Wales, where even my GP observed and commented on my increased stiffness yet still did not suggest appropriate tests. Nor was my dilemma noticed in hospital while recovering from hysterectomy despite a nurse commenting that I had not changed position for hours.

I share your belief that delayed identification and treatment of my PMR has resulted in slower recovery although GCA and LVV have not been diagnosed during my seven year struggle. I am only now finding out about the consequences of such long term use of steroids to my own body, having developed painful polyps throughout my digestive system, whether caused by PPI use or not using PPIs I’m not sure.

I felt at the time that my GP had not dealt with a case of PMR before and either went with the textbook approach or took advice from a colleague, but he started me on 50mg pred ! The tapering regime he proposed led to a flare and a return to higher doses again after six months but luckily by that time I had found this forum and knew a lot more about my condition and how to live with it. This journey has been so much easier than it might have been without the great people who contribute to this forum, bless them all. 🙏

[123-go]

Firstly, Lewis is doing a good job in his choice of study which is, for others interested in knowing, the discrepancy in diagnosis and treatment for PMR/GCA between the North and the South (I may well have not got the title right). It appears that this is needed so thanks are extended to him.

Thank you for sharing your own experience here. Others will relate and feel they are not alone; we learn so much from each other.

I’m so sorry to hear that you are in pain. It would be difficult to say definitively what has caused your digestive problems ie, the steroids or PPIs themselves which are said to often be the culprit.

The example you’ve given re your starting dose illustrates the fact that not enough is known about PMR and its management. GPs are sent/have access to information and guidelines but the question is do they read them and remember them when a patient presents with symptoms?

I’m so glad you found this forum and received reliable guidance from our experts. We have a lot to be thankful for. Best wishes as you go forward on your journey.

[Katiecatweazel in reply to 123-go]

Hello, my hairdresser insisted that I should see my GP after I asked her to be very gentle with my painful head. The GP put me on steroids and unfortunately it was 5 days before I could have a biopsy (negative). I was referred though to a Rheumatologist with a big ego. Under his ‘care’ my CRP/ESR went up unchecked over a period to mid 70s. He assured me (in writing) ‘these things very often resolve themselves’. I disagreed (in writing) and as I was feeling so lousy booked a private appointment with a consultant. However the previous evening I had a dissection of the ascending aorta and damage to the proximal arch etc etc. I’m afraid I have not forgiven the jolly chap. When he saw me after the Emergency op at King’s he said ‘you look like you have been through the wringer’.

I have survived a further 12 or 13 years solely because of King’s College Hospital who luckily for me picked up the pieces.

All the best.

[PMRpro Ambassador in reply to Katiecatweazel]

I hope you pointed out he helped put you there!

[Katiecatweazel in reply to PMRpro]

Hello. Yes I pointed it out, too nicely perhaps to be defined as naming and shaming, to various medical people when giving my history but a veil always came down.

[123-go in reply to Katiecatweazel]

Something in writing may well not have been shut down so easily. In your shoes I don’t think I could have forgiven such an unprofessional comment and instead continued with an element of blame in my heart - but then, I’m horrid 😠! However, there comes a time when there needs to be a line drawn under events or, in your case, non-events. My very best wishes to you.

[123-go in reply to Katiecatweazel]

It comes to something when the hairdresser has more nouse than a trained medical ‘professional’! I can’t put in writing exactly what I think of that rheumatologist as I would be thrown off the forum! The truth for most of us is that when we are feeling really ill or in awful pain we put our trust in our doctors: we don’t have the mental or physical energy to ask questions. Often, it’s only afterwards that we realise that things weren’t handled at all in the way they should have been. Your gratitude for the care you subsequently given must be boundless. Thank you for writing about your experience.

[Katiecatweazel in reply to 123-go]

123 Go you are spot on in your reaction, it’s a pity we aren’t at our best on these occasions! This Forum has clearly shown the wonderful support there is available from people who are also dealing with GCAPMR, LVV.

With best wishes.

[Kafkaontheshore]

Hi 123-go. I too have LVV. My rheumatologist recently told me that one of their patients was admitted as an emergency and needed an aortic repair. Histology showed widespread inflammation. The patient was on no treatment as was thought to be in remission and had no symptoms and inflammatory markers were not raised!

It is clearly a challenge to accurately know whether the disease is active or inactive. I don’t know if this case is unusual. My motto is to be vigilant and know my body and how LVV effects me whilst not being super vigilant/paranoid!!!! Hopefully science will catch up at some point to allow better surveillance.

[PMRpro Ambassador in reply to Kafkaontheshore]

You'd hope someone would publish to spread the word. There was great surprise at one finding that in a patient thought to be in remission on Actemra I think - and a paper published to the effect that you couldn't assume the disease wasn't continuing in the background. I think too many of them forget that the best they can hope for is symptom management, they have cured nothing. And that even if they think the pred is too high or used for too long, it is serving a purpose in - one hopes at least - damage limitation. It would be interesting to know if patients like me who have never tolerated risking too low a dose actually do better long term in terms of the adverse effects of unmanaged inflammation.

[Kafkaontheshore in reply to PMRpro]

Yes fair point. I find it frustrating in the 21st century that we can’t track this disease better. It’s also worrying.

[PMRpro Ambassador in reply to Kafkaontheshore]

There simply hasn't been a biomarker identified that is reliable and specific. I keep telling them that it isn't a new treatment that is needed most, it is a definitive diagnostic and monitoring marker. Pred used well and not in fear can do a great job - and all healthcare systems can afford it but most of the problems are actually caused by the doctors misuse and mismanagement.

[Kafkaontheshore in reply to PMRpro]

Yes but there is a balance to be had isn’t there between managing the disease process as effectively as possible and avoiding steroid toxicity and the morbidity that can offer!! Tricky

[PMRpro Ambassador in reply to Kafkaontheshore]

I am quite sure that used well and carefully that there isn't anything like the toxicity and morbidity that some doctors claim. Matteson published a retrospective study that found that apart from cataract, the so-called steroid adverse effects were found to the same extent in an age-matched cohort NOT on pred. We say a lot that most steroid side effects can be mitigated or avoided altogether for the vast majority of patients. But doctors rarely give any advice about diet or how to manage pred effects. And the way they go about tapering the pred dose creates many of the problems.

[123-go in reply to Kafkaontheshore]

Hi, Kafkaontheshore. It’s good (but not😉) to hear from another member who also has LVV.

Was it really necessary for your rheumatologist to tell you a less than encouraging story about another patient?

I agree that monitoring LVV is challenging. There have been brilliant advances in imaging techniques including non-invasive ultrasound: if only this were available in every hospital with trained operators able to interpret the images! But then what? Unfortunately there is no cure for LVV but surely with knowledge comes power and therefore enablement. I do waffle on-apologies 😵‍💫.

Take care of yourself and please, if you are not confident in what is recommended for you as an individual-protest and ask what alternative is available.

[Kafkaontheshore in reply to 123-go]

I have a good relationship with my rheumy to be fair. Yes, monitoring of LVV needs to be improved. As PMRpro said biomarkers are lacking, especially when on IL-6 inhibitors, and perhaps everyone is too focused on getting off pred far too quickly.

Take care now

[123-go in reply to Kafkaontheshore]

Pleased to hear you have a good relationship with your rheumy- makes so much difference.