Dealing with a relapse after 3 1/2 years.

[hwigston]

I'm gutted. 3 1/2 years ago I lost the sight of one eye to GCA and since then I have been following a strict regime to reduce the pred. I have followed a strict diet. gentle exercise, plenty of rest and managed to get down to 6 mg 2 weeks ago, then bang for no apparent reason I had a relapse. GP thought it very unusual after 3 1/2 years but put pred back to 20 mg for one 1 week and then reducing by 5 mg each week until I am down to 5 again. Have others experienced such a relapse after such a long period and what is the best way to deal with the lower doses? GP seems a little vague about this aspect of pred and basically said I would have to test it and see. I personally would not like to suffer the consequences of trial and error as he is suggesting. The pain, adrenal fatigue, side affects of yet another term of the high dose of pred. Any suggestions would be appreciated.

[polkadotcom]

Sorry to hear you have had a relapse, but it does happen. Hopefully the 20mg will flatten the inflammation again. In most cases of flares it usually is possible to taper down again more rapidly than in the first instance, but with your problems I would feel better doing it at a much slower pace. This would give a much smoother ride down than a jerky step-by-step approach but the end result would hopefully be more settled.

Do hope you are beginning to feel better, you have all my sympathy.

[hwigston in reply to polkadotcom]

Hi. Yes 20 mg did the trick. All the pain has cleared up. I'm only left with light-headedness and fatigue, which I put down to the increase in pred. When I get down to 10 mg I will certainly be insisting that my GP gives me a proper reduction plan which includes 0.5 mg of pred.

[Green_girl]

Wishing you a steady return to normal - I don't have GCA, but my Dad did and it was awful. But want to just add my best wishes and good thoughts.

[hwigston in reply to Green_girl]

Many thanks for your good wishes. I am feeling much better thank you. I feel as though I have just come out of a very fast spin of the washing machine, light-headedness etc.

[jinasc]

hwigston

Yes, I had two flares and both times I had to take 40mg to get it under control again. Both times I was able to reduce more quickly than at first.

However I note you were at 6mg when it flared. 10mg and 5 mg can be a sticking point. The current recommendations, after many years, is that reductions should be no more than 10% at a time once down to 10mgs.

7.5mg and below is classed as a low dose and the side effects lessen and this seems to be the point when your adrenal glands wake up slowly. There is a pattern that can be used, if you want a copy send me a pm.

One thing to bear in mind is that sometimes with GCA you are on a low dose for the rest of your life. I was warned at the beginning that I could possibly be on anything below 5mg. However when I got down to 2mg (after 5 years) my Rheumatologist thought it had gone into remission but was reluctant to take me off those last 2mgs. My hospital was one of those who where taking part in the Tabul Study and the new machine showed it had gone into remission. So I slowly dropped by 0.5mg every month till I got to zero.

[hwigston in reply to jinasc]

Hi sambucca. Many thanks for your very informative reply. I would certainly like to have a copy of the recommended pattern you mention when reducing pred. I am also curious to know what the Tabul Study is. I am aware that I may be on a low maintenance dose for the rest of my life, but rather that than be totally blind. Thanks again for all your info.

[hwigston in reply to jinasc]

Hi Sambucca. I have just googled Tabul Study. I was diagnosed and treated for GCA in Spain and both methods of diagnosis was used, biopsy and ultrasound. The biopsy was the deciding factor.

[Polywotsit PMRGCAuk team member in reply to hwigston]

Hi there - the TABUL study stands for Temporal Artery biopsy v ULtrasound. It is focusing on the diagnostic stage of GCA and whether ultrasound could be as effective as biopsy in diagnosing GCA. I hope it is because to be honest, the biopsy is pretty rubbish! Too many negatives when the consultant is convinced it is GCA anyway.

[estherdevers in reply to jinasc]

Please send me a copy of the pattern. Many thanks

[raymck]

Hi, You have got me worried now, as I thought I was just about ready to quit the Preds after a month on 1/2mg [i.e.1 every other day]. It's not going as well as I had hoped, as after a year & a half on the steroids & sight loss of 1 eye, I was thinking I had paid my dues & all would be well. However, seeing the posts on here makes you wonder, when some days go better than others, etc. No doubt we all react differently to complete withdrawal, but I for one do not even wish to contemplate going back up to a sizeable dose, just to make things feel easier. Recently, I was asked to fill in a spot for a big band tour in Germany & couldn't believe how well it went, so a lot seems to be down to mental attitude it seems & my wish is that you will try to avoid negative thoughts & just press on as normal, as that can do the trick!

Hope you get over the present difficaulty & keep us all posted as to your progress. We shall be watching! Regards,

raymck

[hwigston in reply to raymck]

Hi. So glad to hear that all is going well for you. I have certainly done my best to be positive over the past 3 1/2 years. I have carried on with my cycling, doing a tour of Denmark & Germany earlier this year, and prior to my relapse I had been cycling in France. For me life has carried on and I have battled through the bad times. I certainly didn't wish to increase the dose of pred to 20 mg but when your sight is at risk there is no alternative, at least I will be able to taper dose more quickly to 10 mg when hopefully GP will be able to give me some advice. Good luck with your band.

[jinasc]

raymck

Your wrote " but I for one do not even wish to contemplate going back up to a sizeable dose, just to make things feel easier."

Currently as pred is the only medication that stands between total or partial loss of vision, I don't think you would even contemplate not going back up to a sizeable dose.

It is nothing to do with "making things eaiser" - it is to do with preservation of vision.

Until there is known cause and cure and/or friendlier medication that controls and saves vision loss we are stuck with prednisolone and all that comes with it. Luckily nobody that I know has ever had all the side effects.

I am sorry that I got "you worried" but that is the current situation and will be for the forseeable future whilst researchers are working their socks off to find cause and a cure.

I do not have negative thoughts but I do face reality and take precautions my sight is very precious to me.

I sincerely hope your GCA stays in remission.

[Whittlesey]

Hi Hwigston, I saw the thoracic sugeon (for dissecting cartoid and vertebral arteries) yesterday. He said the GCA can flare at any time. They work with the prednisone and methotextrate to hopefully keep it from happening, he said, but it can happen. Currently, they do not know the cause.

I have been through two flares since I was first diagnosed in 2012. I commiserate on the dealing with the higher levels and then the lowering and almost instant Cushing's type symptoms, fatigue, muscle pain, etc. am sorry this has happened.

At Hospital for Special Surgery in NY, they are currently doing tests of a biologic, which deals with and inhibits the growth of killer t cells which after the first instance, seems to be a problem for those people with GCA. Does this cause a "flare" -- ? I don't think they know yet. Because I am taking methotextrate which can cause immune deficiency, they do not want me to participate in this study because in addition to inhibiting the growth of killer t dells, the biologic can cause immune suppressive problems. I agreed. This may be one substitute for long term episodes of GCA, instead of the prednisone. It would seem to me, that there has to be something instead of prednisone which does lead to adrenal dysfunction, for awhile. If they can replace stem cells, it seems they should be able to come up with something aside from predisone for these inflammations. will try and find the link on these studies with human subjects at HSS in NYC. Hope you feel better.

all my best, Whittlesey NYC U.S.

[Whittlesey]

Hi Hwigston, still searching, but I found this and will include the link:

"...The most recent study, by Hoffman, et al, was a multi-center study with a tremendous flaw: it used alternate-day steroids, which are known not to control GCA.[3] The study by Spiera, et al, could be faulted because of underpowering and, in some patients, steroid tapers that were too rapid.[4] However, the most methodologically sound study - by Jover, et al, in Spain - was the only one with positive results and merits our attention.[5] Among their 42 patients, methotrexate was maintained for 24 months; relapses were seen in 45% of those on MTX , compared to 84.2% of those on placebo.

Currently, the compromise is to get out as fast as is safely possible once the disease is under control, i.e. within six months to a year. If people either have too much toxicity from the steroid or a maximal steroid dose does not adequately control the disorder, weekly doses of methotrexate (7.5-20mg/wk) or azathioprine (2 mg/kg/day) are often employed. Thus the front-loading with the steroids can control the disease and prevent blindness, but adding or switching to an alternative drug can help reduce the severe side effects of long-term high steroids...."

link:

hss.edu/professional-condit...

Article also says:

The Future

learly, other new medications will be tried. The pathobiology of GCA tells us that it is an inflammatory disease in which cytokine profiles in biopsy specimens contain the T lymphocyte products interferon-gamma (INF-gamma) and interleukin 2 (IL-2) and the macrophage products IL-1beta, IL-6, and transforming growth factor beta. These findings all lead us to believe that MTX, azathioprine , one of the TNF inhibitors, or other more specific blocking agents as they come along, may be useful. Indeed, a study looking at etanercept (Enbrel) and infliximab (Remicade) for this disorder is underway.

Until we develop a much more focused therapy, such as a biologic agent chosen specifically for this disease, as opposed to a shotgun like steroids, my recommendation is that when you can't get out after a year of steroids, or you know the disease is very active and will require a lot more steroid for far too long - which can only be discerned from the personality of the disorder as you follow it - it may be time to add another agent that can help you taper off the steroid.

same link. Article is by Stephen Paget

Will look for the link on HSS in NYC

Would also say that one of the doctors in the first pp, Dr. Spiera, (the one whose study was "flawed") I saw him, for treatment, right after the diagnosis, when I was pretty "oh my god!", in NYC. still trying to understand what was happening. He teaches at Mt Sinai, who misdiagnosed me for four very dangerous months, neurologist and my primary care doctor at Mt. Sinai. I could have lost my eyesight. He was one of the "specialists" I was sent to -- after -- they finally did the biopsy, as I had requested. He is close to 80 in age, a very nice man. But he clearly wanted me better and no liability anywhere. He was a nice person, but I felt I wasn't getting all the answers, nor direct answers concerning what I was suffering with. It was always vague, "well, you never know. " and "you feel better don't you?" I wanted to know what I was dealing with. I always felt I was being told to be a good little girl, (I am 60) take the medicine and don't ask questions. After 20 years working in Manhattan as a paralegal, this just was not the answer for me, when I was -- pretty frightened, did not want to lose my sight, nor deal with a four year morbidity.

Did a lot of research and I have my sight and since the initial diagnosis, many very good doctors have said that there is no absolute on the morbidity. I am so very sorry that you have lost your sight in one eye. My mother almost did with GCA. very sorry. You are amazing in your strength, insistence, and continuance.

I will continue to search for this study at HSS. But as the last quoted pp says, they are looking at biologics for GCA because they know what prednisone presents.

all my best to you, in continuing health. hoping you are feeling better,

Whittlesey U.S.

[Whittlesey]

Hi Hwigston,

I found this link which has good information. Medscape is a reliable and often used site. Because I have other large vessels involved, aside from the temporal arteries, I am going to see about the last biologic mentioned, Tocilizumab (TOC). Will talk to the rheumatologist about using this instead of methotextrate. The thoracic surgeon said that if the inflammation stays down, the other large vessels arteries may be all right and not dissect. This may be a good biologic to help that happen.

I hope you find this link and information helpful.

medscape.com/viewarticle/75...

Some pertinent paragraphs:

"...Although there is no clear rationale for GCA being a B-cell-mediated disease, the anti-CD20 antibody rituximab (RTX) was used in a patient with glucocorticoid-dependent GCA. [23] However, as the well responding patient received concomitantly CYC, the extent of RTX efficacy is difficult to judge.

Interleukin-6 may be a key molecule involved in GCA pathogenesis. IL-6 is found to be increased in the serum of active GCA patients and correlates with disease activity. [24] Temporal artery biopsies revealed also increased local IL-6 production. [25] Moreover, IL-6 is a regulator of acute phase response and body temperature. [26] Conversely, IL-6 may also have protective properties such as stimulating angiogenesis. [27]

Tocilizumab (TOC), a humanized monoclonal antibody directed against the interleukin-6 receptor shows good efficacy in rheumatoid arthritis but also in systemic diseases strongly associated with systemic inflammation such as Still's disease. [28,29] We recently treated three glucocorticoid-dependent GCA patients with TOC. [30••] All three patients were stable and free of symptoms but could not taper prednisone to less than 20mg/day without recurrence of disease. We administered six monthly TOC infusions (8 mg/kg bodyweight) and concomitantly reduced prednisone to 7.5 mg/day or less. All three patients showed no further relapse during treatment with TOC and no patient experienced adverse events. Moreover, systemic inflammation as measured by C-reactive protein and serum amyloid A (SAA) levels fell drastically after treatment with TOC was commenced and follow-up PET scans showed strong reduction in large-vessel inflammation. ..."

wishing you good health

all my best, Whittlesey

[hwigston]

Hello Whittlesey. Many thanks for all your replies. What fascinating information you have found. Since my relapse 6 months ago I have at long last managed to persuade my GP to refer me to Rheumatologist. My appointment was last Tuesday, and he was rather horrified to hear that I had had such a problem in being referred. However the outcome was that I have to have a Cortisol test to see whether my adrenal glands are functioning after being on steroids for 4 years. If they are not I will have to take methotextrate to help reduce them. The consultant also has arranged for me to have Physiotherapy sessions something that should be been arranged 2 years ago, and lastly he gave me a 24 hour phone number to ring if I have another relapse. Many thanks once again for your messages, I'm afraid the wheels of the NHS seem to turn a lot more slowly here in the UK. Good Luck and best wishes Heather.

[Whittlesey]

Hi hwigston, posting this link I found from Christine Northrup who is a physician and lectures across the U.S. specifically on women's health and issues specific to women's health. I have found it helpful for the Cushingoid symptoms I suffer from the large amounts of prednisone I have taken.

drnorthrup.com/womenshealth...

And the siberian ginseng and licorice root, if anyone wants to take them would be taken when the prednisone has been stopped. The idea is that they are both gentle adrenal stimulators, which may help to get the adrenals working when the pred has stopped.

I have been through it, twice, the "upping " of the pred and afterwards it is pretty difficult.

I hope you feel better.

sending all my best, Whittlesey NYC U.S.