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Cindy M
03-03-2010, 04:06 PM
I was diagnosed in Jan09 and I have tried a few different drugs but have had no great response to them. I started with Methotrexate - it didn't work, now I am on Methatrexate 150 mg a day. I had 2 IV treatments of Rituxan on Aug 6/09 and Aug 20/09 and have seen no improvement. The only thing that seems to keep this disease somewhat manageable is predisone. I am down to 40 mg a day but I am really noticing an increase in symptoms. When I was on a high dose of preds I would see my ENT every month to month and a half and now because of the increase of sinus problems I have to see him in 2 weeks. I am also experiencing alot more pain with the sinus's. I was really hoping that rituxan would be the answer but it does not seem to be. My Rhuemy said that my WG is the thicker more clustered type of the disease which is harder to treat. Has anyone else heard this same thing.

Jack
03-03-2010, 07:02 PM
Have they tried you on Cyclophosphamide? It was the original treatment for Wegener's together with Prednisolone and was the combination that had an immediate effect on my symptoms. I know that a lot of people get scared at the thought of some of its potential side effects, but Wegener's can kill you too! ;)

Sangye
03-03-2010, 09:42 PM
I agree with Jack. The fact that your Wegs can only be contained by remaining on high doses of pred typically indicates the main engine drug isn't strong enough (eg mtx) or isn't the right one for you (eg Rtx). Sounds like you probably need to give ctx a try. You can't stay on high dose pred for this long. It's extremely damaging and is not sufficient to contain Wegs.

I don't know about your rheumy's statement about the clustered type. It does seem quite plausible, though. Different types of involvement do respond better to one drug or another. Wegs with more "vasculitis" respond better to rituxan than Wegs with more "granulomas". Both are present in Weggies, but some tend to have more inflammation vs more tissue destruction.

elephant
03-03-2010, 10:30 PM
Hi Cindy M, the prednisone won't keep it under control. I have severe sinus involvement and still see a ENT every eight weeks, because of damage. Even when I was on 40 prednisone like you, I still had sinus involvement. Like Jack stated, you might need Cytoxan. Many of us on this forum have to try different drugs to find the right one to get WG in control.
It sounds like your WG is very active. Increase pain and seeing your ENT every two weeks is a sign you need something else quick before it attacks your lungs or kidney or any other organ. Hang in there, and be persistent. Hope you feel well soon.

Cindy M
03-04-2010, 04:13 PM
I had an appointment with a different Rheumy today, I also saw him in February (mine has been away since Jan and will be back in April). Didn't get the greatest news but then again with the way I have been feeling I wasn't expecting good news. In Feb my CRP went up to 12.1 from 8.9 and today it was down to 9.2. My ESR in Feb was the same as Jan at 22 but today it was up to 25. I still don't quite now what all this means. They also found traces of blood in my urine so I had to go for another urine and blood test. The next drug they are looking at me trying is Remicade which I would take along side with methotrexate. I had been on Methotrexate at the beginning, 1st with pills and then we tried injections but they didn't work. Has anyone tried Remicade? It is a Biologics which is a new class of DMARD. My preds has also been increased from 40 mg to 60 mg a day. I just can't seem to get below that 40mg.

pberggren1
03-04-2010, 05:33 PM
Remicade is the same as Rituximab isn't it?

Cindy M
03-04-2010, 06:16 PM
Not as far as I know. The information I have is that it is a Biologics which has 3 different types of drugs that are currently being used. They are: Infliximab (Remicade), Etanercept (Enbrel) and Adalumimab (Humira). As with other drugs there are side effects. I will have to go for a TB test, chest x-ray and viral hepatitis screen prior to starting.

renidrag
03-04-2010, 09:20 PM
Cytoxin is the answer along with prednisone. I started at 80 mgs pred and am still on Cytoxin although I hope to be off this month. Usually a six or seven month ordeal according to most at this forum.

renidrag
03-04-2010, 09:23 PM
oops. I should have reread previous more carefully. Cytoxin is a six or seven month ordeal usually. Prednisone can go on for a long time, although at lower doses. Both are usually prescribed at diagnosis.

elephant
03-04-2010, 10:29 PM
Cindy if it is the RTX IV infusion, that is a good drug for WG. If you have to continue to up your prednisone that means the other immunosuppressive drugs are not working to keep your Wegeners in control. The Rheumatologist that I see is a Wegeners specialist at the Cleveland Clinic in Ohio and they are the ones who know what drugs are more powerful to keep WG in control.
My lab values don't move that much, so I would not get to concerned. Your lab values did not move much. They go by how your feel, Chest x-rays, urine test to check kidney....ect
Hang in there Cindy.

Sangye
03-05-2010, 01:51 AM
Remicade is NOT Rituxan/Rituximab/Rtx.

Remicade/Infliximab is a biological agent and monoclonal antibody like Rituximab, but works quite differently. It's being studied as a treatment for Wegs, but to my knowledge none of the Vasculitis centers are using it outside of studies. It does look promising--especially for "refractory" (ie, non-responsive to other drugs) systemic Wegs-- and may be the next Rituximab.

Remicade is an antibody to Tumor Necrosis Factor (TNF) which is made by the body. The role of TNF is to destroy malignant cells before they multiply and become larger tumors. So the obvious risk of Remicade is that you've taken away the body's Tumor Cop.

I searched PubMed and couldn't find any studies later than 2008 (other than 1 or 2 case studies). That probably indicates long-term studies are in progress right now. Maybe those of us who see Wegs specialists can ask our docs about Remicade at our next visits.

This 2008 paper is by Carol Langford at Cleveland Clinic
Drug insight: anti-tumor necrosis factor therapies... [Nat Clin Pract Rheumatol. 2008] - PubMed result (http://www.ncbi.nlm.nih.gov/pubmed/18506159?itool=EntrezSystem2.PEntrez.Pubmed.Pubmed _ResultsPanel.Pubmed_RVDocSum&ordinalpos=6)
"Experience with the use of anti-TNF agents in patients with Wegener's granulomatosis or giant-cell arteritis has emphasized the crucial role of randomized trials in determining whether a treatment is effective, even in the face of promising preliminary data. Caution is necessary in clinical practice until such data become available."

A 2008 paper.
Novel therapies for anti-neutrophil cytoplasmic an... [Drugs. 2008] - PubMed result (http://www.ncbi.nlm.nih.gov/pubmed/18416584?itool=EntrezSystem2.PEntrez.Pubmed.Pubmed _ResultsPanel.Pubmed_RVDocSum&ordinalpos=9)
"Safety concerns, notably of infection and malignancy, were common and need to be explored in subsequent trials. In addition, concomitant immunosuppressants and non-standardized definitions were major limitations, and future studies of these and newer agents must follow agreed standards of study design and reporting to facilitate clearer interpretation of the circumstances (e.g. disease stage, severity or organ involvement) under which these agents perform optimally. Consequently, use is still limited to centres experienced in such agents and mostly in the context of clinical trials."

This 2008 study includes 1 Weggie but the abstract doesn't say how s/he did:
Anti-tumor necrosis factor therapy: 6 year experie... [Isr Med Assoc J. 2008] - PubMed result (http://www.ncbi.nlm.nih.gov/pubmed/18548981?itool=EntrezSystem2.PEntrez.Pubmed.Pubmed _ResultsPanel.Pubmed_RVDocSum&ordinalpos=4)
"Infliximab and etanercept have been included in the Israeli national list of health services since 2002 for rheumatoid arthritis and juvenile idiopathic arthritis, and since 2005 for psoriatic arthritis and ankylosing spondylitis. The regulator (Ministry of Health and health funds) mandates using fixed doses of infliximab as the first drug of choice and prohibits increased dosage. For other indications (e.g., vasculitis), anti-tumor necrosis factor therapy is given on a "compassionate" basis in severe refractory disease."

Cindy, I have no idea why your docs would be discussing Remicade when they haven't tried Ctx or Rtx--both proven therapies. I'd push for answers on that one if it were me.

elephant
03-05-2010, 02:26 AM
Thanks Sangye, one of my doctor friends told me she has a patient that takes Remicade and is doing well. That is the only person I ever heard of taking that drug for WG. I am so happy to see the Carol Langford did that study. Makes me smile! How are you feeling Sangye?

Sangye
03-05-2010, 02:39 AM
That's great to hear. I wonder if that patient is unable to take anything else. I hope it works for them.

Cindy M
03-05-2010, 06:51 AM
Hi Renidrag, I am currently taking cyclophosphamide (150 mg) per day along with 60 mg of preds. and have tried methotrexate at the beginning.

LilyPony
03-06-2010, 06:38 AM
Wegs with more "vasculitis" ... Wegs with more "granulomas"

Sangye - I am curious about this and haven't read anything about it yet. My tests came back showing windpipe was irritated, sinuses were irritated, larynx oddly normal and perfect. But nothing that "looked" like wegs to the ENT. Perhaps he was looking for granulomas or just a war field, I don't know. There wasn't even anything he felt was even remotely worth a biopsy. Does that appearance sound characteristic of more of a vasculitis type of case? I'm still in the process of trying to be seen by the right people. I"m on board with some prednisone for the next two weeks to control the "allergies" or whatever it may be.

JanW
03-06-2010, 07:29 AM
Lilypony: you indicated on another thread that your doc said that the area would look "diseased and gray" vs. "pink or red" if you were having WG related problems. I have never heard this. The area of scarring around my windpipe is so old it's not even pink anymore, but pink or red was precisely what he was/is looking for in terms of active inflammation. He also found granulomas in the first two rings of my trachea (now sent for biopsy) so I had both inflammation and granulomas there.

And of course, since he says it didn't look like WG to him, it really begs the question -- how much has he seen, or did he confer with pictures on the web like we all do. If he hasn't treated many WGs patients, I would assume later. And anyone who will tell you that WG will be limited over your lifetime to one or two places in your body is being overconfident. It MAY be, but like my ENT says, you have to be humble in the face of this disease - -more is unknown than known.

elephant
03-06-2010, 07:33 AM
Jan you are absolutely right! WG attacked my kidneys and joints first and slowley worked it's way to my lungs and sinuses. I will state this again, WG too me has no limits....

LilyPony
03-06-2010, 08:43 AM
Thank you Jan. I do not know how much experience he has but he sounded quite confident that it looked nothing like wegs, so perhaps he's seen a few cases that were horribly visible. He's considered by many to be the best ENT in my area. We're not far from the medical schools of UNC at Chapel Hill and Duke University, so there's a wealth of doctors here. And he's been highly touted as the best. But if he doesn't have experience, then he can only be useful to a certain extent. Perhaps the blueish gray coloration is sign of other kinds of disease?

I'm taking this whole a thread totally off topic here! Now I am about 8 hours past taking my first 40 mg dose of prednisone and slowly starting to get stuffed up again. I've also got a backache and knee pain. I'm wondering if always aching but I'm too busy to notice. Back, knees, and ankles usually hurt quite badly when it's stormy but I've always attributed that to mild arthritis from the various injuries I've gotten from "trying to be an athlete" as my husband puts it. I've never noticed it in nice weather though and it's beautiful out today. Is it possible that my pain was temporarily relieved by the prednisone and now I'm just noticing it coming back, or is that somehow an effect of the prednisone, or just pure coincidence. I'm trying very hard not to become a hypochondriac!

Sangye
03-06-2010, 08:44 AM
I agree with Jan and Elephant. It's not always as straightforward as actually seeing diseased tissue (assuming he even knows what to look for!).

My nose is a good example right now. The outer part has been increasingly painful for almost a year, the cartilage is loose and the outside of my nose is irregular in color. Occasionally I have pain on a small area of the septum, along with very small infrequent crusting, but nothing else inside the nose. I know this is Wegs but since the tissue looks pretty normal, they can't really call it nasal involvement. My docs concede something is happening there. Hopefully it can be contained by the rtx before it worsens.

I think the "vasculitis" vs "granuloma" distinction can only be made by a Wegs doc. It depends on how the Wegs acts in different areas as well as over time. That's my understanding, anyway.

LilyPony
03-06-2010, 08:49 AM
Sangye - Gosh I have this uncanny ability to hijack threads, don't I? You say that your nose is irregular in color on the outside. My biopsy confirmed involvement is the lacrimal gland area of one eye. That eyelid has a small area just above the eyelashes that looks kind of bruised all the time. The entire eyelid is ever so slightly darker than the healthy eyelid. It looks like I have a little eyeshadow on the one side. Of course after makeup, it's not noticeable at all. Is that similar to the discoloration you have on your nose?

Sangye
03-06-2010, 09:06 AM
(LOL-- the ability to digress from thread topics is a requirement in this group! :D )

My nose is redder than the surrounding skin and is a little splotchy looking. If I'm pale or cold or hot, it gets VERY red and hot. Definitely looks and feels inflamed.

JanW
03-06-2010, 09:36 AM
What's really funny guys is that my nose too is a different color (like a sunburn) on the part that would normally not be covered by sunglasses. I always chalked this up to regular sunburn (figuring the nose is more sensitive) and I'm black so it's not particularly noticeable. BUT, I don't really burn anywhere else on my face. Wonder if this is all part of the WG puzzle (and as you guys know, my nose saddled about six months ago (this is discoloration is kinda good for hiding that, though!). And my crusting is minor too -- my collaspe all happened from the outside (didn't even know that was possible).

Sangye
03-06-2010, 09:39 AM
My Wegs doc said my nose would probably never look "normal" again. It doesn't really look abnormal unless it's pointed out.

elephant
03-06-2010, 11:09 AM
So were talking about discoloration. I noticed that my cheeks ( not butt cheeks) have been red in the morning. They get less red during the day, what is that about?

Sangye
03-06-2010, 11:24 AM
I have that, too. After a shower my face looks and feels extremely sunburned for at least 30 minutes.

jola57
03-06-2010, 07:43 PM
Same here, after a bath I look like a well done lobster, me cheeks are red as well as if I was running a fever. After a couple of hours it goes away

Cindy M
03-27-2010, 05:00 PM
Hi Sangye, I have been on methotrexate, cyclophosamide and rituxan and none of them seemed to work so this is why they are looking into Remicade. I am probably having the wrong attitude here but I am getting so frustrated with everything that right now I don't care what I am on, not even too worried about the side effects, I just want something to work and to be off these preds.

Jack
03-27-2010, 06:55 PM
Getting off Pred is an understandable wish, but most of us don't seem to achieve it. Or pehaps the ones that do stop posting?

elephant
03-27-2010, 11:47 PM
I was wondering if using three or four immunosupression medicines in low doses would work to get WG in remission.

Jack
03-28-2010, 12:35 AM
Before the combination treatment of Steroids and Immuno-suppressants they were both tried as individual treatments, but had a very low survival rate. However, drugs are developing all the time so I'm sure one will come along some time that can replace steroids or a new Immune suppressant that does not require them.

Sangye
03-28-2010, 12:41 AM
Cindy, you definitely need a Wegs specialist to treat refractory (non-responsive) Wegs. That's what I have, too, though we're hoping rtx will work. (Rtx works best for refractory Wegs) The studies on Remicade were not encouraging, so it's not considered a useful drug for Wegs. There are a few other drugs that have some hope for Wegs, so I'm wondering why your doc isn't looking at them.

I also think a Wegs specialist is needed to sort out signs/symptoms of active disease vs damage and side effects. It's not as straightforward as it seems.

I understand your frustration. Really. Did you call or email the VF?

Cindy M
03-28-2010, 04:02 PM
I know 60 mg of preds is a high dose and it's not good to stay on it to long. My question is....what if any is a safe dose to be on? Also what is the highest dose anyone has been on. I am just trying to feel me way around where I stand with the high dose. Also, is there hope to be off the preds or is that wishful thinking. If anyone is or has been in remission are you then drug free or do you still take some drugs.

Jack
03-28-2010, 07:11 PM
As with several members on here, I started out on 1000mg IV, but only for a few days before dropping to around 60mg. I'm afraid that there is no safe dose of Pred. Some doctors say that 5mg is safe because it is similar to what the body produces naturally, but they are wrong, it is not the same thing at all! I developed several serious side effects while still on 5mg.

I have been in solid remission from Wegener's for around 20 years, but only with the help of many drugs. I am now suffering far more from their side effects than any remaining Wegs damage, but I probably would not be here at all without them. ;)

pberggren1
03-28-2010, 09:48 PM
Those are tough questions Cindy.

There is no safe dose of pred. But unfortunately we Weggies have to take it and some of us will never get off of it completely. I think I heard of someone being on 250mg for a few weeks.

I was in remission with no drugs from August 2006 until October 2007. That is the only time I was not on any meds - though I stubbornly quit the Imuram in August of 2006. So maybe that led to the flare I had in October 2007?

elephant
03-28-2010, 10:48 PM
I was told by my Rheumatologist that there is no safe dose of prednisone. I will be on it for life, until they find something better. Anytime I go lower than 10 mg my symptoms of clogged ears and stuffiness increase. I am on 10 and have less stuffiness and less clogged ears, by not 100% better.

JanW
03-29-2010, 12:42 AM
Echoing what others have said, Cindy -- there is no safe dose of pred. I'm probably one of the few members here not on it and not in remission, but my doctor will not put me on it unless I start flaring more severely than I am now. I would feel better on it (and he knows that) but am controlling my joint pain with tylenol for now while I try to work up to a therapeutic dose of mtx, or some other immunosuppressant. I haven't had any increase in my 'classic' WG symptom (which for me are nasal crusting, SS and saddle nose) since diagnosis, and I've had the SS for over five years (now fixed; SS and saddle nose also don't track severity of disease). So while I am uncomforable and in pain with my ankles and back, it's not unbearable. I was given steroids for my misdiagnosed asthma several times over the last five years and ended up with serious osteoperosis. While I knew it was damaging to your bones, I had not idea that such a short period of time in very lose doses (not even 20 mg daily) could do such damage.

Cindy M
03-29-2010, 03:04 AM
Hi Sangye, I'm not sure if you are talking to me or the other Cindy. When you asked if I called or emailed the VF (who or what is the VF) the answer is no. I am seeing a rhuemy who is and has treated WG before. I also see a ENT and opthomologist. I have seen a kidney specialist and have an appointment to see a urologist. I know that my rhuemy has taken my case to other doctors to discuss the best course of action.

Cindy M
03-29-2010, 03:07 AM
I have also done 3 days of 1000mg IV last July because I was not proactive with my symptoms and waited to long before I made the call to my rhuemy. Really took a big step back and have alot of residual effects from the damage. You reallyl are damned if you do and damned if you don't with these meds.

JanW
03-29-2010, 04:17 AM
Cindy - VF is the Vasculitis Foundation, and they can hook you up with docs who specialize in vasculides (of which WG is one). Your local doc can also consult with one of these docs by phone if travel is out of the question. I'm sure that any on here would say that it's very important to have a rheumy who is well experienced with WG in particular to oversee your care, if at all possible. It's not enough to say that they see a lot of lupus, RA or sarc, which are all very common rheumatic diseases. WG is something special all it's own, and because it can kill us, and because there's no protocol because it's so rare, it's important to that your rheumy knows what he is doing.

I have the name of an opthamologist who specializes in rheumatic diseases as well, because they need to understand the potential problems of WG and the eyes.

And personally, I would not have an ENT do surgery on me (and I will have to have this for my SS and saddle nose in the future) who hadn't done many WG patients. That could potentially be a recipe for disaster.

Sangye
03-29-2010, 11:28 AM
CindyM-- I think it's really important to have the regular input of a Wegs specialist who's treating TONS of Weggies, not just some. Your rheumy is likely getting input from others with his same level of experience with Wegs. He may be a great rheumy, but it's not enough with Wegs.

I lost a lot of time with docs who weren't Wegs specialists, including my Mayo Arizona doc who had treated some Wegs (and was in contact with Mayo Rochester at times), He wasn't at all like going to JHU and seeing a true Wegs doc. I wouldn't be in the shape I'm in if I'd had a Wegs doc from the beginning.

Cindy M
03-29-2010, 03:28 PM
I have an appointment with my rhuemy on April 15th and will be asking alot of questions. Thanks for the info.