GPA with right sided pseudo tumour ,leptomeningeal thickening,peripheral neuropathy.

[Gary L]

Hello to all from 57 years young GPA victim.
story started 2 1/2 years ago on vacation to Spain for intended 2 months.Fter 5 days hospitalised with severe ear/headache and right eye closed and swollen.Intravenous steroids brought vision back and following morning ears punctured to lease poison with grommets fitted.10 days later after mri and ct scans admitted to Granada neurology hospital ,further medication 100 mg a day prednisone and lumber test to get me fit to fly home.
6 months later diagnosed with GPA,,tried 7 infusions of cyclophosphemide with no success and transferred to Rhitixumab.
I have now slowly lost my vision due to high steroid damage and the pseudo tumour along with lung function deterioration and what appears to be the normal side effects of both the disease and meds.
I was wondering if anybody else has the same side condition of the pseudo tumour and vision problems.
would like to hear from anybody that can give any input.
cheers Gary

[Pete]

Gary L

I don’t have the symptoms you describe. Hope yours subside soon.

Where are you being treated? Several people on here goto Addenbrooke’s and see Dr Jayne there. They all seem to be doing pretty well.

[Gary L]

Hi Pete,
Thanks for input.
I am lucky enough to be under the Rheumatology Hospital at Royal United Bath.
A purpose built new wing to the main hospital with a team very familiar with GPA and other
autoimmune diseases.
It seems my condition is quiet rare even amongst our already rare group,hence the reason to try and find
people with like for like symptoms.
cheers Gary

[richard052018]

Gary: what was the basis for your GPA diagnosis (ANCA-C, PR3, Biopsy)? Did any of the medications help your lungs? Do you have kidney involvement? What are your inflammation numbers like now? Any chance the tumor can be removed?

I hate to hit you with a ton of questions but in these unique cases, knowing as much as possible really helps.

With regards to your vision, how is your optic nerve looking? Have you had Spine/Brain MRI to rule out other Auto-Immune issues. I did see I'm guessing they checked your lumbar fluid for MS.

Best of luck to you sir. I know you are happy with your doctors but from the outside you are not getting the results needed from your treatment. If I was in your shoes I'd pursue alternatives (if possible, I don't know UK health). Very rare that CTX and RTX can't battle this disease. So sorry for you.

[Gary L]

Morning Richard
They diagnosed from both anca and pr3 .No kidney involvement yet and the lungs have only started to be an issue these last 4 months.
I am waiting for an echocardiogram to determine any link with the breathing Issues.
My an a levels have come right down ,the pseudo tumour is not like an ordinary tumour in the sense it is inflammation of the tissues and fluid but not actually a growth.It mimics all the symptoms of a tumour which is good in one way but not in the other as it’s hard to treat.
ive had both mri,lumbar and at present my ophthalmologist opinion is the extended high level of prednisone 80mg a day for 18 months along with the pressure from the pseudo tumour has damaged the lenses.The optic nerve is holding up.
Unfortunately ,all the different symptoms at diagnosis caused confusion and I’ve learnt there’s no real common Ground between patients, plus the fact it’s not widely common.
Thanks for your input ,I hope I’ve provided enough details ,but still new to discussing this in the open.
cheers Gary

[seied]

Make sure to ask your doctor to treat the side effects (such as bone loss) caused by long-term, high dose usage of prednisone.
I'm not sure that 80mg/day for 18 months is within treatment protocol. You may want to get a second opinion.

[richard052018]

I'm with seied on your prednisone usage. It seems a bit excessive. I would think with the trouble you are having you'd be a good candidate for the plasma exchange therapy, bring that up with your doctor.

I too was diagnosed with ANCA-C, PR3, and classic symptoms, and lungs were my most noticeable presentation. I responded VERY WELL to the Prednisone. I started at 80MG: myself. I've never heard of anyone starting at 100MG a day.

I truly feel for you Gary. I hope you find the magical treatment that works. So many of us have lost so much to this disease. You have had a rough go.

[annekat]

Hi, Gary, I just wanted to say welcome to the forum, haven't been on here much lately and just noticed your posts and the replies. I started out in 2008 with a severe ear infection in both ears, and my dx was unfortunately delayed until 2011, as I was treated for allergies and recurring sinus infections until it went into my lungs. So my similarities to your case ended pretty much with the initial big ear infection. Cyclophosphamide helped quickly with the lung involvement that got me into the hospital and dxed. I have never been on Rituxin, am currently on methotrexate and a small amount of prednisone. I was initially on 50mg. of pred and quickly tapered down over a couple months to around 20-30mg., where I stayed for awhile. I agree with others that you are on a very high dose of pred for a long time. I have heard of maybe one or two others who took that much over some months. it would be very hard on me with my small bones and hereditary osteoporosis. But every case is different and I don't know enough about yours to argue against what your docs are doing. Just wanted to say you have come to the right place, as there are many knowledgeable people on here, and with unusual symptoms as you have, it is always an interesting challenge to help solve the puzzle of your particular case. Of course it would be better if you didn't have a reason to be here, and I'm hoping for some answers to some of your questions. Keep us posted!

[Alysia]

Welcome to the forum, Gary. I think you must get second opinion from the best experts possible.

I live in Israel but my WG dr. Consults with prof. Jayne in Addenbrook. I tag @Geoff (https://www.wegeners-granulomatosis.com/forum/member.php?u=364) and freakyschizogirl who are his patients. I tag also @gilders (https://www.wegeners-granulomatosis.com/forum/member.php?u=7952) who might be able to help.

When my wg flared in my eyes I was lucky to find an eye dr. In the near city who just came back from UK after working for years in London in an eye hospital at it's vasculitis unit. So you might try this one too.

Sending you prayers. Please update us.

[freakyschizogirl]

Hi Gary

Being tagged by Alysia I feel duty bound to reply :).
I am under Addenbrookes and would say easily that Dr Jayne is the top bloke to see or have your doctor's consult with, especially with your rarer complications.
I do not share your symptoms but I will agree with a few others that 100mg a day of pred seems very excessive and you don't want to risk further damage unnecessarily.
I first went to Addenbrookes as my local rheumatologists didn't have a clue how to treat auto immune disease like this but they like to take a punt at it and I wasnt confident they were prescribing the right medications for me.

Give Stella a call at Addenbrookes on 01223 217259 - she is the best nurse you will ever speak too.

If you need to know anything re Addenbrookes please don't hesitate to send me a private message.

[gilders]

Hi Gary and welcome,

I think the long term, higher-than-usual dose, of Pred could be due to the fact that the Drs had no other ideas of how to control the pseudo tumour?

Long term steroid use usually effects the eyes in the form of glaucoma or cataracts. You say your optic nerve is ok, so I presume no glaucoma. Cataracts damage the lens, is this what is wrong with your lens, or other damage?

Where exactly is this pseudo tumour?

I have been a member of this forum for many years and we have had MANY Wegener's patients from all over the world pass through over the years, but I don't recall any pseudo tumours. If your lens damage is not cataracts, then I would be concerned that along with the unusual pseudo tumour, you may have something going on other than GPA/Wegener's.
From past experience, it seems too easy for Drs to blame a rare, complicated illness, such as Wegener's, for a whole host of illnesses, before they rule out other possibilities. I.e. if you had turned up at hospital with only the pseudo tumour, would they have diagnosed Wegener's, or would they have a different diagnosis?

I am also treated at Addenbrooke's (I arranged a transfer from Bradford, via my GP) and would recommend them. The problem at the moment (with COVID) is getting access to any kind of appointments, never mind a transfer/referral. Although Bath have experience of dealing with Wegener's, do their other specialists. I have seen ENT, lung specialists and eye specialists that do specialise in Wegener's at Addenbrooke's.

There's a good chance that Bath do know what they are doing and if unsure, they're likely to be in touch with Prof. Jayne, so try not to worry. But the one thing you must do is make sure that they have a plan for you. I think US hospitals tend to be proactive in telling patients what they are doing, what they plan to do, giving many details of tests and investigations. Sometimes in the UK it seems as though they expect you to just accept that you are "in the best hands", don't worry, take your meds and we'll update you when necessary.


Keep us all up to date with your progress and I hope everything settles down.

[Geoff]

Hello Gary,

Sorry to read about your plight but welcome to this Forum and the best source of information and help!

Freaky has beaten me too it (not for the first time!) and given you all the info I could possibly pass on. We are both fortunate to have come under the wing of Dr Jayne and count our lucky stars. I agree with Richard with regards to Plasma exchange therapy. It was given to me for 3 consecutive days on my first admission to Addenbrookes after DX back in 2008 and set me on the road to recovery.

None of us walk the same road with regards to this illness so its a battle to find out what works for you. We are fortunate to have RTX in our armoury when you think about how the treatment regime was not so many years ago.

Wishing you the very best.

[Gary L]

Hi Anne,
Thanks for your message.Its good to hear from so many people.
i hope life is treating you kinder now and you managed to reduce your meds right down.
Will keep you informed if anything drastically changes.
Gary