PMR with a twist?

[Bittebitt]

I'm still a mystery to the specialist. I have PMR, That's clear. Was diagnosed last october. The mystery is that my blood tests don't fit properly. It all started in may 2015, with a persistent pneumonia. Took me three courses of antibiotics to get through. The last one together with 40 mg prednisolone. (Tapering each third day).  The infection markers were still high, and my gp decided to do some further blood work. Result: bone marrow affection of some sort. Thrombocytopenia, counts decreasing every time I test. Nettopenia. Not bad, but could make me susceptible to infections. Monocytosis, made the haematologistssuspect a sort of leukemia: cmml. Very nasty. Now, the monocytes are normal, which has made the haematologists abandon that that theory. I've also been through several  bone marrow tests. The last one on thursday april 21. Waiting for the result. All the earlier tests have showed that it could be either reactive changes caused by rheumatic disease, or it could be caused by some malignancy. 

[Bittebitt]

Nettopenia should be neutropenia. Sorry. 

[piglette]

What are the results of your blood markers? 

[Bittebitt in reply to piglette]

If you mean ESR and CRP, they are normal now. I'm on 10 mg prednisolone still. The abnormal tests are other markers that might indicate either an inflammatory disorder, or a disease in the bone marrow. Sometimes, PMR is masquing other, more serious conditions. That's what they have been looking for in me for almost nine months now. I think I'm in good hands. I will probably be sent to the norwegian national hospital for further investigation in a short time. 

[PMRpro Ambassador]

PMR isn't the disease - it is the name given to the symptoms of some underlying disorder. The PMR we all talk about is what is left when other causes are ruled out and even then may not be correct.

I hope you are under a real vasculitis specialist - I think a "chat" with Keyes on this and the Vasculitis UK forum would be useful for you.

[Bittebitt in reply to PMRpro]

Hi PmrPro. 

Thank you for your answer. I'm norwegian, so I'm not quite sure if I understand what you are saying about PMR not being the disease. In fact, I hope that it is, but I'm still being followed up by haematologists and rheumatologists, due to abnormal bone marrow and blood test results, that might be caused by either a rheumatic inflammation (Pmr) or a myelodysplastic disorder. Of course,I hope for PMR, myself. It's 11 months exactly since I came down with pneumonia. After almost six weeks on antibiotics, I still had ESR 82and crp 52. My GP put me on 40 mg tapering dose prednisolone. So far as I remember, the typical Pmr pain started when I had come down to five mg Prednisolone. I then told the GP that I suspected PMR, which he told me to administer Prednisolone myself, because he said that I (as an expirienced reg. nurse in rheumatology) knew the disease better than him. At that time, even though esr and crp was creeping down, other blood tests were nor normal, so my GP sent a request to an outpatient specialist clinic on haematology. I've been through four bmp's. Three of them came out inconclusive, (either reactive or myelodysplastic), still waiting for the result of the fourth. It' s a lot for me to cope with, but I will folkow your advice and contact Keyes after a while. Sorry, this is a lot for you to read. What I didn't understand in your answer, was: Why couldn't this be PMR with atypical blood tests? I' m aware that malignancy can mimic PMR, but I certainly hope that isn't the case with me😕

[PMRpro Ambassador in reply to Bittebitt]

PMR is the name given to the symptoms we experience, poly many, myalgia painful muscles - which are caused by another unrecognised illness. As you say, malignancy is just one reason for having the symptoms but there are plenty of others including rheumatoid and other inflammatory forms of arthritis. The PMR we talk about here is almost certainly due to an autoimmune problem that makes our immune system attack the blood vessels, causing inflammation of the vessel walls so they swell and affect the blood flow through them as the diameter of the centre narrows. It is a vasculitis as opposed to an arthritis - it just leads to arthritic and rheumatic symptoms. It responds well to pred. It is important to rule out the other possibilities because while pred is the only drug that works for us, in other cases there are far more effective ways of managing the symptoms and, in some cases, preventing longer term damage to other tissues.

In GCA is is larger arteries that are affected. In PMR it is most probably what is called the microcirculation - the very small capillaries in the muscles that supply the oxygen and nutrients to the muscle fibres. Without enough oxygen and other nutrients even mild exercise becomes like running a marathon or running up stairs and your muscles get very tired and sore as the recover - which takes much longer than normal.

It COULD be PMR with atypical blood tests - but obviously haematology have found things that concern them and are search for the link. In some cases, "PMR with atypical blood tests" has another name - it could be lupus or it could be spondyloarthropathy or something else. The PMR you experience is a symptoms of something else and I suggested you speak to Keyes as she has investigated just that: she was diagnosed with PMR originally but she was sure it was something more and eventually was proven to be right. She has a diagnosis and is being treated with other medications - pred wasn't the right thing for what she has.

[30048]

Oh goodness....you really threw me on that reply. My understanding has been that PMR is a manifestation of Giant Cell Arteritis. I do have a question for the pro.  I was just told that I am "pre diabetic" and am also losing kidney function due to my four plus years on prednisone. It MUST" be reduced.  I know that many on this forum have been on it longer than I .....other than trying to taper have you had any course of action on those fronts?

[PMRpro Ambassador in reply to 30048]

If you have GCA and have PMR symptoms, which sometimes happens but not always, then yes, it is a manifestation of the GCA. But you can have PMR in its own right with only the muscle symptoms - or you can have something in between where larger arteries in the chest area are affected but the arteries in the head are not involved in which case it is unlikely you will develop what are described as the "typical GCA symptoms". GCA doesn't JUST affect your vision and cause headaches - it can be all over your body. And see my response to Bittebitt.

I've been on pred for nearly 7 years. I'm not pre-diabetic although I was Cushingoid 4 years ago. I rarely eat any quantity of carbohydrate which helps there (almost no bread, no cakes, pasta, rice), I have lost 36lbs of "Cushingoid" weight. I live in Italy - the doctors at my local hospital don't approve of using "steroid sparers" in GCA or PMR as they don't think they work and other than methotrexate there are no recommendations in the most recent guidelines for PMR. I haven't seen the latest for GCA, don't know if they are out yet.

It's all very well them saying it "MUST" be reduced - if the GCA returns they have to manage that or risk your vision. Pred is still the mainstay, there is nothing yet to replace it although tocilizumab looks very promising but it is not yet approved in the UK for use in GCA. Currently there is a consultation ongoing to not recommend is uset. Seems slightly mad and very premature since we are waiting for the results of the clinical study using it in GCA. But then, it does cost about £12,000 a year. Pred is rather cheaper. Perhaps their idea of cost-effective and ours are a bit different.

[Bittebitt in reply to 30048]

PmrPro

Thank you for your answer. You're really doing a great job👍

[30048]

Thank you PMR pro for your analysis .  My case is complicated to say the least, by my age ...almost 80 , the fact that I have had my optic nerve leak twice,... stenosis in my spine, ....nerve pain from my bout with shingles  and IBS .  It is like walking a tightrope trying to manage all the symptoms . My eyesight has taken precedence .there is still debate on the PMR .  It is a clinical diagnosis I realize and because my pain is in my back and legs ....it may just be the stenosis.  Your diet recommendations  are  hard due to my intestinal problems  but I will try and adjust  as much as I can.  I am honestly exploring medical marajana as it is legal now  and  . does seem to work for some people.  It is a bit scary as I have never taken drugs like that but I am really at my wits end,

Thanks again for all your help and if I get to Italy again.....maybe we can have a spritz together

P.S my own gut feeling is that the GCA is throughout my body and is a contributor to the butt paint as well as the carpal tunnel syndrome.  Prednisone is the only thing that works