Younger age GCA data?

[CloudGazer6]

I've just bought the new collection of articles by Dasgupta and Dejaco, 'Polymyalgia Rheumatica and Giant Cell Arteritis', published by OUP last month.

I was very disappointed to see on the first page of this brand-new book that the authors state in the 'Preface' that PMR and GCA are 'exclusively occurring in older people'. This is a book directed ostensibly at health-care professionals, and presumably a large part of its intended audience includes those who are not rheumatology specialists. I can't help thinking that there is a missed opportunity here to have highlighted the broader age spectrum that these conditions can affect. So often I read here on the forum that people at the younger age range are being told 'you're too young' to have PMR or GCA, or at least how it makes the diagnostic pathways more challenging for the doctors.

I'm not an 'older person' (51), either in my life stage nor under the usual medical criteria, which are usually related to retirement age or people seen under gerontology specialties whose medical needs and presentations can be different to those with younger bodies - although I concede that I'm older than 30, older than 40.

Does anyone know if any age-related data is being collected for these conditions? If not, I might take this on as a project when I'm well enough to do so (there is potentially scope for me to do this my work). It seems to me that there are considerable socio-economic implications as well as medical factors (eg atypical presentation) that could perhaps inform medical decision making in the future, and help patients work with their doctors to achieve successful outcomes.

[PMRpro Ambassador]

There are some papers in the medical literature that warn quite clearly about this attitude - that there ARE cases of GCA in under 50s and there is a risk of them being missed because of this mindset that if you are under 50 it cannot possibly be one or the other. With PMR it could, I suppose, be said that it matters less, except that I believe there are countless younger women with PMR. who could have their life returned to them if they hadn't been fobbed off with diagnoses of depression, somatism, CFS/ME or fibromyalgia. Or even ignored totally as I was in my very early 50s.

The patient representatives on the committees have fought to get the age in the guidelines reduced - at least it is now over 50 not over 55 as it was. But the experts seem to fall into two camps - one of which is adamant they don't appear in under 50s (of course they haven't seen it, they don't look for it and if they do, deny it exists) and the others - who I get the impression are generally a bit younger and not so well established - who are quite happy to say, yes, it can happen. I spoke to a few at the OMERACT meeting a couple of weeks ago who say yes, they have patients of that age range.

What is even worse is the use of the statistic "average age at diagnosis" for PMR - 73! Plenty of us well under that - but not diagnosed.

I, for one, would be delighted if you took it on!

[CloudGazer6 in reply to PMRpro]

Thanks very much for this, PMRpro. Already planning my first data sets!

[Runrig01 in reply to CloudGazer6]

I too totaly agree with everything you say. I spent 14 months struggling with stiffness and pain because my rheumatologist felt I was too young at 47. 18 months after symptoms started I presented with many classic GCA symptoms, and spent the next 2 years arguing my case. Ended up getting referred to the experts for diagnosis. So I for one feel it would be a great project, and would be thrilled if you took it on. I don't want anyone to experience the horrendous journey I'm still on. I have areas of my tongue that have turned grey and the tissue is abnormal, due to vasculitis affecting my tongue. Now seeing an oral medicine expert in 10 days time. I do feel if I'd been taken seriously it would not have progressed to this. Good luck xx

[PMRpro Ambassador in reply to CloudGazer6]

Eeeh- that'll keep you out of mischief!

[CloudGazer6]

Or make it...!

[PMRpro Ambassador in reply to CloudGazer6]

Either way...

[piglette]

A while back the World Health Organization defined 'middle-age' as being 45-59 years, 'elderly' as being 60-74 years and the 'aged' as over 75 years of age.

One foot in the grave at 50!

[paddyfields]

'exclusively occurring in older people'. The simple addition of the word "almost" would probably be enough to save the day and allow medics to entertain the idea of cases outside the "norm". Young onset Parkinsons, variously said to be under 40 or under 50 often suffer for years before a referral to the appropriate neuro.specialist and a dx is made and yet 5/10% of patients with this disease, the second most common neuro disease, are under 40 and there are cases of children one as young as 8 years. If only they would listen carefully to what their patients are telling them. As the saying goes "if it looks like a duck, quacks like a duck , it probably is a duck."

[PMRpro Ambassador in reply to paddyfields]

Yes, I'm a great believer in the duck theory...

[Jackoh]

Sounds a great idea- well overdue. Look forward to hearing your progress.

[Jenlouh]

I am 42 and diagnosed with PMR by my GP. I am seeing the rheumatologist on Tuesday, so will let you all know what his views are. My GP hasn't questioned the fact I'm under 50 and diagnosed me on symptoms and blood results without taking my age into account. My days would be very different without the diagnosis.

[Caro12line]

Dasgupta is my Rheumy. I personally would argue with most of what he says. Also he does not practice what he preaches.

I presented with head pains, biopsy negative. He said " your biopsy is a clear negative you don't have GCA, which is good news because you wouldn't want that". However pain returned when Pred was reduced so I asked for further tests (actually to tell the truth I was in so much pain, I pleaded!) and an Ultra scan showed up something, so he sent me for a PETCT. And.... GCA of Aorta and Subclavian arteries diagnosed.

After doing my own research I questioned the diagnosis and asked Do I have Takayasu? He said I was too young and not Asian so I can't have that, but it wouldn't matter because the treatment is the same. I have noticed that after that question, on my notes and blood forms it now says LVV (Large Vessel Vasculitis) and not GCA.

Sorry for the rambling, my point is I think age or ethnic origin should not wholly be considered in a diagnosis. I think this is more important in the future as ethnicity is increasingly becoming mixed and age is becoming just a number.

[PMRpro Ambassador in reply to Caro12line]

"He said I was too young and not Asian so I can't have that"

Hmm - really! This is a quote from Johns Hopkins:

"The “typical” patient with Takayasu’s arteritis is a woman under the age of 40. There is a 9:1 female predominance in this disease. Although the disease has a worldwide distribution, it appears to occur more often in Asian women.

Takayasu’s arteritis is a rare disease. The best estimates of the disease frequency suggest that 2 or 3 cases occur each year per million people in a population."

and on the same page in front of me I have references to "the third non-Asian patient in the English literature", as well as an abstract from a 1995 paper (so it isn't recent stuff) which says " Although TD (Takayashu's disease) appears to be more common in Asia, increasing numbers of patients of different races are observed in Western countries. ... We show here seven Caucasian hypertensive patients with TD and renovascular stenosis and arterial hypertension."

So experts say Caucasians and under 40 - but you are too young? And there could be, what, say 15 a year in the UK at a very conservative estimate? Generally it is said that it can be TA up to 50, GCA after - and one lady said her dx was changed on her notes from TA to GCA when she reached 50.

And while the treatment is currently basically the same as GCA - I suspect the monitoring is a bit different (can only be more!). There is currently an NHS consultation about using tocilizumab in Takayashu's and GCA - being recommended for Takayashu's but not for GCA.

[CloudGazer6]

Thank you very much for this. Very interesting and food for thought

[Polywotsit PMRGCAuk team member]

What we need is for the precise relationship of PMR and GCA and the ageing process to be discovered and properly understood. I think what rheumies who spend a lot of time researching PMR and GCA are concerned about is the very many mistaken diagnoses of PMR or GCA when there is potentially something more permanent going on. Large vessel vasculitis is one of the possibilities.

[PMRpro Ambassador in reply to Polywotsit]

I'm not sure that is the real answer though - I don't think that relationship is particularly important. What does need to be learnt is that a TAB is fairly meaningless if it is negative (fine if it is positive) and that other imaging techniques should be used in less than clear cases with symptoms they say "aren't GCA". The age criterion should be forgotten altogether and a better diagnostic process encouraged.

Not that that is going to happen in the NHS in the foreseeable future. It might if the government does manage to introduce private medicine where the funding follows the patient - but then you will have the same problem as in the USA where the poorly insured are ignored and especially the not-rich elderly don't count.

[Polywotsit PMRGCAuk team member in reply to PMRpro]

Oh yes agree about the imaging. I was thinking in particular about PMR.

[PMRpro Ambassador in reply to Polywotsit]

I don't really think the ageing process has a great deal to do with it - and people with PMR need the imaging too. A lot of us with PMR almost certainly have a large vessel vasculitis as the root cause - but if you respond fairly well to pred they don't look further at the time, or any other time come to that. I've had a flare that is not typically PMR but did have PMR items in it. However, I have a doctor who is happy if I'm at 10mg for a considerable time - and for too many patients that is not the case. There is constant pressure put on patients to reduce and to reduce in the wrong way - look how many people on the forums manage to reduce with my slow approach but have a return of symptoms if they follow their doctor's instructions for 2.5mg or 5mg reductions. The patient is the same.

[CloudGazer6]

I think we might be straying away slightly from my initial thoughts about the age-related data. We just don't know if there are physiological differences in presentation or imaging sensitivity and specificity, for example, even before we think about the experience of the complexities of diagnosing atypical patients.

[PMRpro Ambassador in reply to CloudGazer6]

I was just answering Kate's point really. More imaging is more important than understanding relationships. All they can do is treat the symptoms - and that is to some extent the same for any vasculitis.

No, you're quite right, age-related data is where you start. The problem then will be that there will be lacking data for the under-50s whose doctors are closed to the concept they could have GCA - do you see what I'm saying? We're only seeing the top of the iceberg - there's an awful lot of unknowns...

[CloudGazer6]

I absolutely agree but if we have identified deficits of information then we are well on the way to planning how to start investigating. The we are able eventually to work towards providing and collaborating a source of information for doctors who may benefit from a stronger evidence base.

[PMRpro Ambassador in reply to CloudGazer6]

Exactly...