J-A-N

[j-a-n]

Hi All --- was dx 9yrs ago, GCA then PMR -- 1yr ago RA. Started on 65mg Preds reducing to 25 then follow on down. Started MTX in 13 at 25mg. Had many chest infections. Stopped MTX each time. In15 had a nocturnal cough, sent to Cardioligist for checks. Told I needed an Aotic valve replacement. Stopped MTX again. Had opp Restarted MTX , More chest infections. Liver failure, Took off MTX, 3 mths later put on trial drug, Baricitinib, been on for 2 yrs. Still on Preds 7mgs per day.

[MhairiP]

Oh dear - you've been through the mill! Welcome to the forum - you'll find lots of expertise and support here. If you have any questions, please ask.

[Hidden]

Hi, sounds liked a lots of on off drugs. Hope you feel better after surgery. If you search for methotrexate you will see several. Posts. I am sure you can advise others too. Welcome.

[KellyInTexas in reply to Hidden]

So I have a question. ( maybe this is a two fold question.)

Why would some patients like poor J A N need to be started on 65 mg of pred? I’m guessing the answer is their inflammation numbers were just that remarkably high? Or is it level of immobility due to ,” frozen” inflamed joints/ muscles due to damage/ inflammation at a micro Vascular level? ( a micro- Vasculitis level I believe one of the admins recently described it to joints and muscles...)

The first question and second must obviously link to the other?

So why not start each patient with high inflammatory markers and dibilitating pain on methotrexate? After all, this is an auto immune disease.

Why is methotrexate the drug of choice?

If patients were seen sooner, ( before it got so severe) would plaquinil not help?

What criteria do physicians go by in deciding on treatment? ( I know it’s always best to treat the patient before your eyes... and criteria are only guidelines. An astute , well seasoned doctor knows better than to be hog tied to a criteria list.)

I’m really wondering why methotrexate is used over other medications. Is it the first , “ go to” drug?

[PMRpro Ambassador in reply to KellyInTexas]

Not sure what you mean? The initial diagnosis was GCA - pred is the drug of choice there, 9 years ago there was no alternative in the form of tocilizumab at any stage. There was possibly a bit of suggestion that mtx MIGHT act as a steroid sparer then, by 2013 it was more established and was introduced in the way it was then. It is only recently it has been suggested it may work better used in the first month.

However, only pred reduces the swelling quickly and reliably and the very high doses are used at the outset to reduce that swelling asap to reduce the risk of loss of vision quickly. If there are already vision symptoms or jaw claudication then a high dose is indicated. The blood markers aren't directly correlated - they can be low and you go blind without treatment or they can be high in "just" PMR without any cerebral symptoms. mtx, or anything else, doesn't change that high dose start approach, it would be unethical to risk the patient's sight.

However, here there has now been an additional diagnosis of rheumatoid arthritis. mtx has been the first line approach for RA for over 30 years. It is a DMARD, a disease modifying anti-rheumatic drug, which in some way acts on the actual disease process in RA - I don't think anyone knows how - and reduces the joint destruction. It is more effective than plaquenil - which really has no role to play in GCA either. But I think it can fairly be said that J-A-N has comprehensively "failed" mtx.

mtx is the first in a hierarchy for RA - a proven effective DMARD that has a good price/benefit relationship. Then there is a range of others which should be tried in order as each "fails". There is no point jumping straight to biologics because when they fail there is nowhere to go. Almost all RA drugs stop being effective at some point and another must be tried. But nothing has yet replaced pred - because even tcz only works for about half of patients. Pred works.

[Austin7 in reply to PMRpro]

My wife GCA on 19 July started 60m had bad side effects with Pred after 8 weeks then at 20 m she was in hospital for three weeks and put on meth and Pred 30m then pred 20 for 14 days now back home on meth weekly and is about to taper every 2weeks to no pred at all the doctor thinks meth alone will be ok so is this a recipe for disaster or has it been done before. Thanks david

[PMRpro Ambassador in reply to Austin7]

Couldn't really comment but it isn't an approach for GCA I have ever heard of and it isn't one I would be happy with personally.

However, if the dose of pred isn't enough to manage the GCA the symptoms will return.

What bad adverse effects did she have with pred? Was the hospitalisation because of the adverse effects of retuning GCA symptoms?

[Austin7 in reply to PMRpro]

Acute anxiety and delerium intense shaking . she wouldn’t take the tablets hardly able to walk. No signs of gca. They had her on 30 for two weeks then started meth at 15 and pred at 20 for two weeks and decrease by 5 every two weeks. Their idea is to get off pred entirely and continue with meth. So how and when does the adrenal gland kick in. I just cannot understand any of this.

[HeronNS in reply to Austin7]

When you say "no signs of GCA" does this mean she never really had signs of GCA or that the prednisone has dealt with all her symptoms?

If the latter, GCA is not cured by pred, it is not "cured" by anything. It will go into remission in its own good time, with GCA usually something between 2-4 years, although PMR, the related illness, can last longer. All the disappearance of GCA symptoms meant was that pred was doing its job. Mtx is not a substitute for pred. It is used to help people wean off pred, and also to help them manage with a slightly lower dose of pred. It is often not even successful for those purposes and it is not useful as a substitute for pred. If she has GCA and her doctor is weaning her rapidly down to a low dose and eventually off pred altogether then I fear he is putting her at risk.

A good way to think about the function of pred in dealing with active GCA (or PMR) is to compare it with insulin. Insulin is needed to keep diabetes in check. You would never think of stopping insulin if you are symptom free. You know that the insulin is what is keeping you well. (Unlike type 1 diabetes. of course, GCA will eventually go into remission and pred can then slowly and safely be discontinued.)

To address your specific question, adrenal function should start to return as the pred dose reaches the physiological level of what the body produced normally, around 7 or 8 mg. Even after stopping pred completely it can take up to a year for full function to return. But everyone is different and I suspect, although I don't know for sure, the longer the adrenals have been suppressed possibly the longer it will take for full function to return. I suppose like everything else age will also play a part, but I'm only speculating.

Here is a paper which describes PMR and GCA and includes basic guidelines about safe tapering, remembering always that symptoms are key, not blood results.

rcpe.ac.uk/sites/default/fi...

[PMRpro Ambassador in reply to Austin7]

As Heron has explained, below about 7mg pred the adrenal production of cortisol should start to function to top up the reducing dose of pred to what is required. It tends to be irregular at first but that isn't really a problem until well below 5mg although a lot of people feel very very fatigued. Your wife has only been on pred about 3 months so it shouldn't take long to reestablish normal service - they usually say 1 month for every month on higher doses of pred.

[gifford7]

re: need aortic valve replacement

You may have aortitis caused by GCA. My sister had this: heart murmer led to diagnosis of 5.5cm thoracic aortic aneurysm and bad aortic valve with regurgitation, diagnosed with imaging studies. She had open heart surgery and is now OK. see:

academic.oup.com/rheumatolo...

"Increased awareness of large-artery involvement is paramount because patients with GCA are 17 times more likely to develop aneurysms of the thoracic aorta compared with their age/sex-matched comparators [12], and occurrence of this complication is associated with a significant increase in mortality [13]. In addition, aortic aneurysm/dissection has been shown to occur in one in every five patients with GCA and large-artery stenosis in one in every eight [14]."

"Because of the frequency of aortic involvement, medical and surgical thoracic guidelines have recommended a baseline CTA or MRA in all patients with newly diagnosed GCA to evaluate the presence and extent of thoracic disease [48]."

When I was diagnosed with GCA in 2017 I had an MRA with contrast which showed I didn't have an enlarged aorta.

[jinasc in reply to gifford7]

Both the aorta and pulmonary were checked by my Rheumy every two years. It is in the diagnosis and treatment guidelines which are available to all on the PMR GCA , BSR , NHS and Patient.info site and can be downloaded from all those sites.

I had GCA only - no PMR.

[gifford7 in reply to jinasc]

These guidelines on GCA involving aortic aneurysms/dissections and large-artery stenosis don't seem to be known by many GCA patients and physicians. Of course we would seldom hear back on this website for GCA patient with aortic dissection!!!

My sister was within 12 months of an aortic dissection[rupture] according to her cardiothoracic surgeon who repaired the aortic aneurysm and replaced the aortic valve.

She was diagnosed with GCA with vision loss and PMR in 2010 but never had imaging of the aorta; until 2017 when a routine physical exam noticed a heart murmer; further investigation showed an aortic valve with regurgitation and 5.5cm aortic aneurysm.

Successful open heart surgery in 2018. The referenced article of 2017 covers new information on LV-GCA, coauthored by a well known GCA rheumatologist at the Mayo Clinic, Eric Matteson.

[jinasc in reply to gifford7]

I, personally and some others, over the past 11 years have told and sometimes sent hard copies, of both the PMR and GCA guidelines on Diagnosis and Treatment, issued by the BSR to all Rheumatologists.

Those guidelines are not issued by NICE...............to GPs.

However, they are always on the net.............

Yes, Eric was absolutely a lovely man, he retired earlier this year.

[HeronNS]

What a time you have had, so sorry. How is the Baricitinib working? Do you find it's helping you?