Polymyalgia Rheumatica

Having now had my telephone consultation with my GP, I will ask him to arrange a DXA before dropping the alendronate permanently. He is concerned that the prednisolone and my T1 diabetes make my bone fracture risk greater. The thing is, this is a statistically based decision rather than one based upon my own health and history, with low HbA1c and virtually no diabetes related complications. There is an overall increased risk of fractures that are a lot higher in T1 diabetics. But T1's with minimal or no complications, such as myself, seem to have considerably lower risks of fractures than those with complications. In the papers I have read, probably a bit too quickly, there is a lot of uncertainty about a link between glycosylation of bone proteins and fractures, but the link between bone mineral density and fracture risk for someone like me ssems unclear, statistically speaking. So, the DXA bone check it is, if I can get one, as this should give something useful on which to base my decision on using alendronate to offset the unwanted effect of prednisolone.

EileenH, thanks for digging up the data on stopping the steroid too quickly. My 2 mg/day seems quite low by most experience, then. But I will carry on very slowly reducing the dose until something nasty happens. I may even go to alternate 0.5 mg/day dosing before stopping at the end if all goes well.

Hi steve,

You do have other factors in your favour - gender, age & I think you said you train in the gym and presumably you don't have a family history of osteoporosis.

A dexa scan should be done early on after starting steroids so that a base line can be established and if necessary supplementary treatment such as AA given. My rheumatologist was adamant I should go on AA which I did not want to do as I have oesophageal problems & low Vit D - both of which are contra-indiacted for AA use. I said, 'let's wait until my 2nd dexa', which was due anyway. Report from osteoporosis specialist 'patient does not require treatment with biophosphonates'.

Atypical stress fractures of the proximal femoral shaft have been reported in patients treated long-term with alendronic acid (time to onset ranged from 18 months to 10 years) and there is also the very small risk of necrosis of the jaw. In my opinion, it is a drug that may be necessary for some but should not be given automatically unless there is evidence that 'you' need it.

Very good idea going really slowly on the last couple of mg. That dose is not going to cause many, if any, side effects and some people do find the last couple of mg a bit tricky. Far better to have a couple of extras months on a very low dose than rush it and have to go back up.

Good luck,

Margaret

Kate - am currently on holiday but will look it up when I get home. I tend not to put in references as they delay the comment appearing.

I swear - sometimes I wonder where some doctors put their brains! Anaemia is one of the least problematical aspects of PMR and one I've rarely found in the literature! Patients sometimes develop pernicious anaemia - but there is no proof it is DUE to PMR as such. It is also an autoimmune condition if I remember rightly. Correlation is NOT causation!!!!

I'll go back to my prosecco to relieve the BP implications...

Eileen

The Kirwan group paper that provides recommendations for deciding whether a patient has "pure" PMR or something else that is leading to a constellation of symptoms that would often be described as polymyalgic syndrome and its subsequent treatment with prednisolone is:

Our approach to the diagnosis and treatment of polymyalgia rheumatica and giant cell (temporal) arteritis

V. Quick and JR Kirwan

J R Coll Physicians Edinburgh 2012; 42:341-9

John Kirwan is a Consultant Rheumatologist at the University Hospitals Bristol NHS Foundation Trust.

One paper suggesting the duration of treatment may be considerably more than 2 years is:

Polymyalgia rheumatica. Duration of therapy and long-term outcome. William T. Ayoub, M.D. C.Michael Franklin, M.D., Dennis Torretti, M.D. Department of Rheumatology, Geisinger Medical Center, Danville, PennsylvaniaUSA

suggesting that 40% of patients require corticosteroid therapy for more than 4 years:

The abstract from this paper:

Behn A.R, Perera T and Myles A.B.: Polymyalgia rheumatica and corticosteroids: how much for how long? Ann. Rheum. Dis. 42:374 1983

says

"In a prospective study of 176 patients in whom polymyalgia rheumatica (PMR) or giant cell arteritis (GCA) had been diagnosed between 1968 and 1980 the effect of corticosteroid treatment was studied. In those with PMR alone an initial regimen of 10 mg prednisolone daily and for the majority of those with GCA 20 mg daily were adequate to control symptoms. No patient suffered a serious disease complication after starting treatment. Regular follow-up enabled the minimum effective corticosteroid dose to be used. Complications of treatment were infrequent. Corticosteroid treatment has been withdrawn from 72 patients after a mean of 31 months treatment (range 3-103 months). Thirty subsequently relapsed, all within 21 months of withdrawal. No clinical feature predicted those who were more likely to relapse. No rigid treatment schedule should be used in these diseases."

Irritatingly, I can't find the reference for the paper which gave the evidence for the approximate distribution of patients as 25/50/25 percent which I'm fairly sure was a review paper. I'll keep looking! In it, however, it was said that the 25% who were able to discontinue pred within 2 years were at a higher risk of relapse at a later date than patients who took pred for longer. About 50% were on pred for between 2 and 4-6 years (it was a review paper so included results from several studies which had different time scales) before being able to stop taking it. A further 25% required a low "maintenance" dose for up to 10 years or even longer.

However, the papers I have quoted above all refer to periods of treatment of well over 2 years being not uncommon. Several authors talk about a clear distinction between 2 groups of patients: one group has an uncomplicated progression for usually anywhere from 1 to 3 years and are able to reduce to zero in that time. Others experience repeated flares as they reduce and in the meantime it is accepted by several experts that the primary cause of flares is reducing too fast and the patients who experience little relief of their symptoms possibly do not have PMR alone.

Professor Kirwan at Bristol addresses this problem with his method of first of all distinguishing between patients who have a polymyalgic syndrome which responds well to steroids and those who don't and then reducing the dose of steroid slowly, after first being confident that the initial inflammation present has been well dealt with by leaving patients on 10mg for a year. It has often been noted in the past that the 10mg/day dose poses a hurdle that patients often find difficult. The next is round about 5-7mg/day - and that has been accepted as the point at which adrenal glands that have been relatively inactive are having to start to work again. Anecdotally, several patients on this and the northeast group forum have reported their rheumatologists being keen for them to remain at 5mg/day for anything up to 9 months and those who have done so have then been able to continue their downward path with relative ease.

PMR has few rules - what works for one patient may not work for another. To suggest that it lasts for 2 years and if it hasn't gone by then it is a wrong diagnosis is irresponsible at best and cruel at worst: many patients feel it is their "fault" they aren't better, others are so disappointed by not being well again after 2 years that they enter a spiral of depression. Far too many GPs are reading from that hymnsheet and it is downright unfair. If the Ayoub paper is correct in suggesting 40% need pred for more than 4 years that is a very large proportion of patients is it not?

Eileen

Call me cruel and irresponsible. In reality, if PMR had soecific features, such as a lab test with 100% sensitivity and specificity, this discussion would be moot. In my experience (42+ years practice of consdulting rheumatology), if one hews to a fairly specific set of criteria, then most (possibly all) PMR patients can say good-bye to the disease by two years. When the criteria are broadened (younger age at Dx, lower or normal sed rate, atypical distribution/timing of stiffness, associated joint swelling, significantly + ANA test, etc), one opens the door to other diagnosic considerations. After all, what is the meaning of the word "diagnosis?" Answer: Read The Doctor, His Patient and the Illness, by Michael Balint (or Ballint).

It is quite understandable that a generalist-type physician would like to label illness presentations having SOME of the classical criteria as PMR......but an experienced rheumatologist, or a top-notch internist, should have high INDEX of SUSPICION when the signs and symptoms of a considered disease are atypical. Not doing so, and not keeping one's mind open, does the patient a disservice.

if one hews to a fairly specific set of criteria, then most (possibly all) PMR patients can say good-bye to the disease by two years

.

That may or not be true. I suspect that if you stick to patients that meet specific criteria then perhaps those patients are more likely to behave according to the book. Many patients do not meet specific criteria (myself included). Naturally, I accept that if a person does not meet the specific criteria then other diagnoses should be investigated, but PMR may still be the final diagnosis.

I was investigated for everything in the 2 years pre dx, all negative. I have not had shoulder problems, only my hips and my ESR was 100+. 30mg of steroids and I was walking again within 12 hours. So presumably 3.5 years & 3 flares later, I do not have PMR??

From the American College of Rheumatology:

In PMR, results of blood tests to detect inflammation are most often abnormally high. One such test is the erythrocyte sedimentation rate, also called "sed rate." Another test is the C-reactive protein, or CRP. Both tests may be very elevated in PMR but, in some patients, these tests may have normal or only slightly high results.

PMR affects adults over the age of 50

There numerous research papers that contradict your opinion, e.g.

http://www.ncbi.nlm.nih.gov/pubmed/17131799

Polymyalgia rheumatica is a disorder that affects people over 50 years of age. The etiology of the disease has not been hitherto clarified exactly. Its incidence among people over 50 is in the range of 0.1-0.5%. The incidence rate peaks in the age group of 60-70 years. It is also found in younger people, but far less frequently.....

It should be noted, that polymyalgia rheumatica may also be present if the erythrocyte sedimentation rate and C-reactive protein values are low. This disorder is also characterised by fast and effective response to corticosteroid, which should be administered for 1-2 years. In some individual cases a different dosage regime may be necessary: steroid administered in low dosage over a longer period of time.

Margaret

I am waiting for the next instalment with bated breath. I could add other comments, but I don't think they'd let me.

"Not doing so, and not keeping one's mind open, does the patient a disservice."

Touchee dear sir. You would do well to bear that in mind.

Both I and my consultant husband assume you must be retired by now. If not, you either should be or indulging in catching up on the CPD/CPE you quite obviously have felt for some considerable time is beneath you.

I can only feel extreme pity and sympathy for any patient who has had the misfortune to have you as their care provider.

Netfret,

I'm afraid my reply 'waiting to be approved' is rather boring I suppose. So this is the 'trailer' for it.

I think I was 'endeavouring' to explain that I am both too young and have had it far too long for it possibly to be PMR. I certainly wish I did not have PMR, but I do.

I can't remember the finer details, but it was quoting the U.S. Rheumatology Guidelines which seem to say something rather different from that suggested earlier. Like this, for example.

The American College of Rheumatology has released the first classification criteria for polymyalgia rheumatica - aimed at helping physicians identify patients with this condition, which occurs in persons aged 50 years or older who have recent onset of pain in the shoulders, neck and hips along with other inflammatory symptoms not explained by an alternate diagnosis.

And also details of research showing age distribution I think. Nothing topical, of course.

As an after thought, it did strike me as a little unusual for our erstwhile friend to choose to get involved in a Patient Discussion Forum, but there is no law against it, I'm sure. That aside, it is an inherent part of any forum like this that we do not give 'medical advice' (regardless of our personal qualification).

Long trailer. Sorry.

Margaret

I rather think Trolls Anonymous are at it again.

vco i have read your posts and do not intend to waste my time replying to any that are written from now on carolk

Well VOC, you are either having a laugh at our expense or are seriously out of date and in fact, a danger to your poor patients.

I had, sadly, a rheumatologist who sounds rather like you...... he put me through hell and had no idea of correct methods of reductions of pred. He too told me I would be free of PMR within two years! Well here I am into year three and guess what, still PMR...... do I still want PMR.... NO....... do I still want to be taking prednisolone.....NO. This disease takes its natural course and does what it does..... none of us want to be taking the drugs we are and your comments are far from helpful or supportive. It is this kind of thinking that we come across all too often from the medical profession. When YOU have suffered from the awful pain and disability of PMR, then please feel free to come back and empathise with us. Until then I suggest you go away and educate yourself into what is actually happening today with PMR/GCA and where current research is taking us. Happy reading!

VOC guy

My PMR could not have been more sudden in onset. I went to bed in normal health, and by the following morning I could not lift a cup of tea. 36 hours after starting on 30mg Prednisolone I was virtually pain-free, and five days later (a holiday weekend intervened) my ESR was in the 80's.

So I assume that if you had been my rheumatologist you would have diagnosed PMR, as did mine. He is far from being a general physician, as a consultant rheumatologist at a large NHS teaching hospital. He also has a private practice, and lectures at a university medical school.

But here's where I diverge from your simplistic view. At the time of diagnosis I was 61, and almost 3 years later I am taking 10mg daily. My rheumatologist said at our last meeting that, in line with current research and advice about best practice, he would hope to have me on 5mg a year from now. And we have of course discussed steroid-sparing agents.

The idea that I might be psychologically dependent on steroids is both insulting and laughable. No-one in their right mind would continue the regime unless they had no choice. We are not stupid women, nor are we cowards. We arm ourselves with every latest piece of information as a way of dealing with this intractable illness.

Something I find puzzling is that rheumatologists like yourself do not find this sufficiently interesting to stay on top of current thinking in the hope that some day a clue might emerge.

well said wendy but i do think this guy is winding us up .however you made some excellant points carolk

Good grief, I never realised there was so much controversy about how PMR is characterised or best treated. I'm not sure VOC guy would have recognised my PMR as such. My symptoms occurred quite gradually, starting with a slightly stiff neck and some light-headedness, then pain in my shoulders, later pelvic girdle pain with severe morning gelling, then pain with gelling around elbows and knees, all from about early March to late May just over 3 years ago. My first visit to the doc was very early on in 2010 and as my symptoms were very non-specific, nothing was done and no tests carried out. It wasn't until March 2011, after a load of blood tests that finally showed raised ESR & CRP (can't recall the figures, though not through the roof but sufficient for diagnosis) that prednisolone treatment started at 10 mg/day. Voila! No pain after about 2 weeks, but with quick improvement in 2-3 days. I am T1 diabetic, hence the low dose start. Roughly around the time I had the dose down to 5 mg/day, my endocrinologist arranged another ESR & CRP check, both of which were within the normal range.

Just as a quick update in my own case, if it's of interest or use to anyone else here, I'm booked in for the DXA in a couple of weeks' time so that my GP and I can decide on whether I should continue with the alendronate. I would like to be sure as to whether I should be on this pretty aggressive drug. Prednisolone seems pretty tame by comparison, or at least it should be on my current 2 mg/day dose. I have a supportive GP who is willing to take the time to discuss issues with me and didn't quibble when I requested the DXA. It's a shame that we won't be able to establish a proper baseline for my bone density as the DXA will be done a long time after starting the steroid and AA/Ca/D3 regime. Surely as a matter of course, DXA should be done for anyone soon after starting his or her prednisolone therapy.