Rituxan - didn't work well for MPA

[mrtmeo]

My mom had one iv dose 700mg of cyclophosphamide and then, 4 weekly infusions of Rituximab.
She is almost at the 4 month mark from her first rtx infusion and has not reached remission.
Her kidney function has not improved and she is dumping too much protein.
She also, has an internal bleed which is most likely due to the vasculitis and has had this since diagnosis.
She has lung nodules, ground-glass opacities in the lungs, 4th stage renal failure with high proteinuria and hematuria, feet and abdomen swelling, eye pain, livedo reticularis, cough (developed during treatments), fatigue, weakness, SOB, weight loss and neuralgia in bottoms of feet.

During and after the treatments, it was like she was in a constant flare.
She is still anca positive with high mpo antibodies.

Her nephrologist just put her on Mofetil because she didn't tolerate the Imuran and hoping this will help put her into remission.
I think she would have done better on the cyclophosphamide for treatment instead.
I have not found anyone that only had rtx for MPA, so I wanted to present my mom's case to anyone looking for a treatment.

The only improvements have been no more eye pain or feet swelling.
She is now on 8mg prednisone and 750mg mofetil per day.

[annekat]

I'm sorry your mom hasn't done better. I know a lot of the literature for WG/GPA says remission can be expected in 3 months, but I think that is not really typical, from reading this forum. I don't know about for MPA. I also hear RTX works better for some than others, and may take longer for some than others to get the same result. Has she had another infusion, or are they talking about giving her one? I wonder, too, whether CTX might have worked faster and better for her. It worked great for me, but my problems were not as severe, and I don't have MPA. It is disappointing to read the list of your mom's symptoms and lab results and to hear that they haven't improved. I hope something or someone is able to turn things around for her.

[mrtmeo]

I'm sorry your mom hasn't done better. I know a lot of the literature for WG/GPA says remission can be expected in 3 months, but I think that is not really typical, from reading this forum. I don't know about for MPA. I also hear RTX works better for some than others, and may take longer for some than others to get the same result. Has she had another infusion, or are they talking about giving her one? I wonder, too, whether CTX might have worked faster and better for her. It worked great for me, but my problems were not as severe, and I don't have MPA. It is disappointing to read the list of your mom's symptoms and lab results and to hear that they haven't improved. I hope something or someone is able to turn things around for her.

Hi Anne,
Yes, the usual time frame from the studies for remission is 3-6 months and the studies don't list the patients treated and their individual results.
Some have really good results with cyc and some don't, but I have only seen people with MPA have good results with cyc and not rtx.
I see alot of wegs folks not doing well with the cyc but do better on the rtx.
However, they all had cyc first and then rtx, so not sure if it is the combo or not for weg's.

If I remember correctly, Kaysee had only rtx and it worked well until her 4th month from the first infusion and she had to have another.
I hope she gets into remission soon.

I am sure praying that the Mofetil puts her into remission and stops the kidney and lung attacks.

[annekat]

I've had the feeling most WG people have done well with CTX but RTX has become preferable for the reasons of long term toxicity with CTX. I still hear of people being put on CTX initially, perhaps due to it's quicker working time, cost, and ease of use, if their symptoms are fairly severe. A few people are initially put on MTX or one of the other "maintenance" meds, along with pred, of course, if their involvement is fairly light. Many of us had CTX because RTX was not widely available or easily approved at the time we were initially treated. I've seen a lot of variability in how well people with WG do on RTX, although most seem to be quite happy with it. However, many seem to need repeat treatments with it in order to continue to do well. The lucky ones go into at least a medicated remission for a long time and don't need more RTX or CTX but most of these still need a maintenance med and pred for quite awhile.

Your mom may not go into what her docs would call a solid remission, as many of us haven't, and the use of the word "remission" may vary from one doc to another. But you should at least expect her to show a lot more improvement than she has, to the point where even if some symptoms remain, her lab work looks a lot better, showing that the disease has been slowed way down or stopped in its tracks. I really hope this for her. Then, if she has small or more severe relapses and needs more RTX, or CTX if that should be decided, or an adjustment in her maintenance meds, it would be quite normal and what many of us have experienced. That's why I say many of us have not really reached remission, because we end up having these small flares when we try to taper off the meds, typically the pred. But our overall quality of life has improved vastly due to the successful original treatment. It sounds like your mom could use some opinions from other specialists as to to why she is not responding to treatment and what needs to be tried next. I wish we here on the forum had more answers.

[Rose]

I was told when first diagnosed that cyclophosphamide had to be used because of my kidney involvement. My pulmonologist had hoped to avoid it and just treat me with methotrexate but then my blood word showed that my kidney function was rapidly deteriorating and a biopsy confirmed WG She said that because of kidney involvement they had to bring out the big guns. I was referred to a nephrologist who took over my case and was treated with IV cyclophosphamide and went into remission in about 4 months

I am surprised also that cyclophosphamide has not been tried. My understanding is that cyclophosphamide gets you into remission and methorexate, imuram and cellcept maintain remission.

Rose

And I also had pulsed Solumedrol x 3 days before commencing cyclophosphamide. They really threw everything at me but it worked. I think you need another opinion

I see it has been tried but just 700mg. I think an IV protocol of cyclophosphamide is worth a try to get her into remission or even daily tabs since the Rituximab hasn't worked. I was also on high oral doses of pred which were reduced only once I started to respond to treatment. 8mgs seems a very low dose for someone who has not responded to treatment.

[mrtmeo]

I was told when first diagnosed that cyclophosphamide had to be used because of my kidney involvement. My pulmonologist had hoped to avoid it and just treat me with methotrexate but then my blood word showed that my kidney function was rapidly deteriorating and a biopsy confirmed WG She said that because of kidney involvement they had to bring out the big guns. I was referred to a nephrologist who took over my case and was treated with IV cyclophosphamide and went into remission in about 4 months

I am surprised also that cyclophosphamide has not been tried. My understanding is that cyclophosphamide gets you into remission and methorexate, imuram and cellcept maintain remission.

Rose

And I also had pulsed Solumedrol x 3 days before commencing cyclophosphamide. They really threw everything at me but it worked. I think you need another opinion

I see it has been tried but just 700mg. I think an IV protocol of cyclophosphamide is worth a try to get her into remission or even daily tabs since the Rituximab hasn't worked. I was also on high oral doses of pred which were reduced only once I started to respond to treatment. 8mgs seems a very low dose for someone who has not responded to treatment.

Hi Rose,
My mom was not doing well with the prednisone and it wasn't doing anything for her symptoms but make them worse.
She was given some salumedrol the same day as the cytoxan.
Her biggest problem is the protein dumping in her urine and the ARB(angiostensin renin blocker) is not helping much.
She still has blood in her urine also, indicating the disease is not stopped yet.
I don't know if the Dr will let her get cytoxan because her creatinine has been stable at 2 and hasn't gotten worse the past few weeks.

Because of my mom's lungs, she wouldn't be able to go thru surgery if she needed a kidney transplant and she could never handle dialysis.
At her worst, her creatinine was 3.3 and at its best was 1.8.
Her Neph said he could get her kidney function back to at least 1.2 and would be disappointed if it stayed above 1.5.
There is a kid that has his video on the vasculitis foundation's website that had a creatinine of 9 at his worst and it 1.2 four years later when he did the video. He has MPA and had cytoxan, plasma exchange and dialysis with Imuran for 2 years maintenance.

I wish they could use plasma exchange for people with creatinine of <5.6, so some can have a better chance at recovering their kidney function.

My mom also, has some kind of internal bleed that the treatments have failed to slow down or stop, so she needs Epogen shots and lots of protein to get her hemoglobin up.

[mrtmeo]

Rose,
If I forgot to thank you for your valuable info on the mofetil and Imuran, thank you!

[Wegetarian]

I see alot of wegs folks not doing well with the cyc but do better on the rtx.
However, they all had cyc first and then rtx, so not sure if it is the combo or not for weg's.


My understanding is about the same as Rose mentioned, that if you have a serious outbreak of Weg's then CYC is preferred as it is the fastest way to stop the disease. When I was DX'd last year I only had Weg's in my nose, and was treated with just prednisone at first, and a few weeks later started on MTX. However when I got a flare this summer and my lungs and kidney were attacked they wanted to use CYC IV's. I was still on 25mg of MTX/week which I stopped as we started using the cyclos.

There are some studies on the effectivenes on MTX compared to CYC. Here is one, not too recent, but dunno if much has changed as these are old drugs.
http://www.medscape.org/viewarticle/712891_5

[mrtmeo]

My understanding is about the same as Rose mentioned, that if you have a serious outbreak of Weg's then CYC is preferred as it is the fastest way to stop the disease. When I was DX'd last year I only had Weg's in my nose, and was treated with just prednisone at first, and a few weeks later started on MTX. However when I got a flare this summer and my lungs and kidney were attacked they wanted to use CYC IV's. I was still on 25mg of MTX/week which I stopped as we started using the cyclos.

There are some studies on the effectivenes on MTX compared to CYC. Here is one, not too recent, but dunno if much has changed as these are old drugs.
http://www.medscape.org/viewarticle/712891_5

Hi Weget,
From what I have read in the literature, GPA and MPA can start out mild, meaning it doesn't effect the lungs or kidneys.
This is when they like to use the milder drugs.
However, even when treated, those who flare (that have kidney involvement), usually end up with either kidney attacks around the 2-3 year mark.
This is not to scare people into thinking they are going to lose kidney function, but to keep people on the alert that the possibility of kidney involvement can happen fast, if it does and to be aware by checking cmp and urinalysis or urine dipsticks just incase.

Many never have lung or kidney involvement, thank God.
If the creatinine is >5.6 they will use plasma exchange with cytoxan, but you can't get the plasma exchange unless your creatinine is that high.
I would really have liked to have the option of plasma phoresis to help my mom's kidneys and lungs, but her creatinine didn't qualify.
There is a study underway plexivas to see if it would be good for those with less severe kidney involvement.

How many months were u on the cytoxan?

[annekat]

Hi Weget,
From what I have read in the literature, GPA and MPA can start out mild, meaning it doesn't effect the lungs or kidneys.
This is when they like to use the milder drugs.
However, even when treated, those who flare, usually end up with either kidney attacks around the 2-3 year mark.
I go more by what I read here on the forum than by "literature", since I know the latter can be all over the place, depending on where you read, can be based on studies which may not represent an accurate cross section of the WG population, and can be overly alarmist. My sense is not that people with light involvement who flare will "usually" end up with lung or kidney problems at the 2-3 year mark. Some will. Many, or I would say most, will not. Or if they do, it may take a lot longer than 2-3 years. Everyone's case is different. If most "mild" WG sufferers did follow this scenario, I think we'd hearing about it a lot more than we have. This is not to overlook the unpredictability of WG or to hide from the fact that anything can happen at any time.

[mrtmeo]

I go more by what I read here on the forum than by "literature", since I know the latter can be all over the place, depending on where you read, can be based on studies which may not represent an accurate cross section of the WG population, and can be overly alarmist. My sense is not that people with light involvement who flare will "usually" end up with lung or kidney problems at the 2-3 year mark. Some will. Many, or I would say most, will not. Or if they do, it may take a lot longer than 2-3 years. Everyone's case is different. If most "mild" WG sufferers did follow this scenario, I think we'd hearing about it a lot more than we have. This is not to overlook the unpredictability of WG or to hide from the fact that anything can happen at any time.

I didn't mean that everyone who flares will end up with kidney failure, but that those who have the kidneys become involved usually end up with failure starting at the 2-3 year mark.
I can't find the study that states this right now, but will post it when I find it.
My mom's kidneys started to fail at the 3.5 year mark.

The whole point here is for people with vasculitis to be on their guard at this time frame to keep an eye on kidney function.
Once we lose are kidneys, dialysis is not a great option, but if we can prevent kidney failure, this is the point.

[annekat]

I didn't mean that everyone who flares will end up with kidney failure, but that those who have the kidneys become involved usually end up with failure starting at the 2-3 year mark.
I can't find the study that states this right now, but will post it when I find it.
My mom's kidneys started to fail at the 3.5 year mark.

The whole point here is for people with vasculitis to be on their guard at this time frame to keep an eye on kidney function.
Once we lose are kidneys, dialysis is not a great option, but if we can prevent kidney failure, this is the point. OK, I get it now, and I think maybe you posted the study before. I'm at the 3.5 year mark from beginning of treatment, though I'm at the 5 year mark for actual Wegs involvement including the undiagnosed time. So, yes, this is important to keep in mind. But I'd revise the statement not to say that this group "usually ends up with failure", since if it is caught early enough and treated, kidney failure will most likely not happen. True, if we do not monitor our kidneys and they become involved, it can happen fast, and without the additional needed treatment, we may be quite likely to experience kidney failure and be candidates for dialysis or transplants. Your point is well taken. And it sounds like your mom has needed more aggressive treatment for her kidneys for quite a long time. Which I guess we already knew from reading your posts. I'm so sorry that issue doesn't seem to be getting resolved, and wish her the best for a turnaround.

[mrtmeo]

OK, I get it now, and I think maybe you posted the study before. I'm at the 3.5 year mark from beginning of treatment, though I'm at the 5 year mark for actual Wegs involvement including the undiagnosed time. So, yes, this is important to keep in mind. But I'd revise the statement not to say that this group "usually ends up with failure", since if it is caught early enough and treated, kidney failure will most likely not happen. True, if we do not monitor our kidneys and they become involved, it can happen fast, and without the additional needed treatment, we may be quite likely to experience kidney failure and be candidates for dialysis or transplants. Your point is well taken. And it sounds like your mom has needed more aggressive treatment for her kidneys for quite a long time. Which I guess we already knew from reading your posts. I'm so sorry that issue doesn't seem to be getting resolved, and wish her the best for a turnaround.

Hi Anne,
I will revise my comment, so others don't get the wrong impression.
Thanks for your concern for my mom.
I wish I would of known about the RPGN earlier.

[annekat]

Hi Anne,
I will revise my comment, so others don't get the wrong impression.
Thanks for your concern for my mom.
I wish I would of known about the RPGN earlier. It's OK. It's just a matter of semantics. I was seeing "kidney failure" as meaning really far gone, while you may have been seeing it as including earlier signs of the kidneys starting to fail. I'm probably being too picky. Now I'm having trouble remembering what RPGN is.

[mrtmeo]

It's OK. It's just a matter of semantics. I was seeing "kidney failure" as meaning really far gone, while you may have been seeing it as including earlier signs of the kidneys starting to fail. I'm probably being too picky. Now I'm having trouble remembering what RPGN is.

Hi Anne,
Many go into end stage renal failure at time of diagnosis and after treatment regain their kidney function to near normal or normal.
There is a young guy on the vasculitis stories that had a creatinine of 9 at its worst and settled at 1.2 after treatment.
RPGN = Rapidly Progressive Glomerulonephritis.
My mom has pauci-immune crescentric rapidly progressive glomerulonephritis.
At her worst, her creatinine was 3.3 and at its best was 1.8 but her nephrologist was convinced hers would have went below 1.5.
I am praying it still improves, but it hasn't and her disease is not in remission, yet.

[annekat]

Yes, I'm aware from some of those on here that people can have really bad kidney failure and overcome it. It is quite scary, though. I'm glad your mom's creatinine hasn't been near the "end stage" level but hope you can get some more acceptable numbers before long. The RPGN stuff is over my head and I don't have the time or energy to delve into it at this point. "Rapidly progressing" does sound a bit scary, though. I wonder if she is in the hands of the best and most experienced vasculitis/MPA docs she can get.

[mrtmeo]

Yes, I'm aware from some of those on here that people can have really bad kidney failure and overcome it. It is quite scary, though. I'm glad your mom's creatinine hasn't been near the "end stage" level but hope you can get some more acceptable numbers before long. The RPGN stuff is over my head and I don't have the time or energy to delve into it at this point. "Rapidly progressing" does sound a bit scary, though. I wonder if she is in the hands of the best and most experienced vasculitis/MPA docs she can get.

Her nephrologist has been dealing with vasculitis patients for 30+ years, but I don't know how many he has treated.
I have to ask him.
The local vasculitis chapter leader never heard of him, so not sure that he is well versed in it, but it's all we have for now.
Transferring records becomes a difficult battle and her case is so complicated.
Her neph is definately, knowledgeable in so many areas of drugs and drug interactions, plus he is the most accurate with her records than any other dr she has ever seen.
He is much more knowledgeable than a rheumatologist we saw last year.

[annekat]

Well, I'm not one to talk, since I don't see anyone with a lot of experience. The doc you have for your mom sounds OK and I'm sure knows who to consult with if he feels the need.

[Wegetarian]

I agree that it sounds like a good idea to be on the lookout for kidney issues for wegs who are in remission, its so easy to not notice anything even though there would be a problem with the kidneys. I didn't experience any symptoms, and I guess I was just lucky that I got my blood and urine tested this summer as it apperars the symptom I had wasn't even weg's related and still I was hospitalised a week after the results.

I posted a link to an article where a doc recommended wegs patients to use these dip sticks to measure their urine. I did ask a rheumy and my neph about it, but they weren't convinced its a great idea since those tests aren't too reliable. For me I guess they will do regular urine tests now, and besides with blood and protein in my urine those sticks would be positive for me. But I guess for wegs whose kidneys are working fine it might be good to check monthly and then ask for lab tests if the sticks show positive results.



How many months were u on the cytoxan?

I still am, guess its going to be my fifth infusion next week. They were talking about 6-10 infusions when I was hospitalized, but the latest I heard it would be 6-8 and then a switch to Aza.

How come your mom has such low pred dose? 8mg sounds very low. If she was DX'd in June I take it she has been on pred for less than 5 months.. When I relapsed in July I first got 1g IV's for three days and 80mg pred, which was slowly tapered so that I'm now on 30mg. Guess I will be on 10mg in december, but they wrote that they are using a faster tapering as I've responded well to the treatments, so just curious why your mom seems to be tapered fast even though it didn't sound like the other meds were helping her that much. Last year I was dx'd in August and I think I was still taking 10mg in May even though I felt great. Great if she doesn't need to take much, but just wondering as it sounds like a very low dose.

[Rose]

Hi
My mom has pauci-immune crescentric rapidly progressive glomerulonephritis.

I have/had same kidney biopsy result

Rose

[mrtmeo]

I agree that it sounds like a good idea to be on the lookout for kidney issues for wegs who are in remission, its so easy to not notice anything even though there would be a problem with the kidneys. I didn't experience any symptoms, and I guess I was just lucky that I got my blood and urine tested this summer as it apperars the symptom I had wasn't even weg's related and still I was hospitalised a week after the results.

I posted a link to an article where a doc recommended wegs patients to use these dip sticks to measure their urine. I did ask a rheumy and my neph about it, but they weren't convinced its a great idea since those tests aren't too reliable. For me I guess they will do regular urine tests now, and besides with blood and protein in my urine those sticks would be positive for me. But I guess for wegs whose kidneys are working fine it might be good to check monthly and then ask for lab tests if the sticks show positive results.



I still am, guess its going to be my fifth infusion next week. They were talking about 6-10 infusions when I was hospitalized, but the latest I heard it would be 6-8 and then a switch to Aza.

How come your mom has such low pred dose? 8mg sounds very low. If she was DX'd in June I take it she has been on pred for less than 5 months.. When I relapsed in July I first got 1g IV's for three days and 80mg pred, which was slowly tapered so that I'm now on 30mg. Guess I will be on 10mg in december, but they wrote that they are using a faster tapering as I've responded well to the treatments, so just curious why your mom seems to be tapered fast even though it didn't sound like the other meds were helping her that much. Last year I was dx'd in August and I think I was still taking 10mg in May even though I felt great. Great if she doesn't need to take much, but just wondering as it sounds like a very low dose.

Her Dr took her from 40mg pred down to 20mg and then, 15 days later, took her down to 10mg because it was causing high bp, sugar and osteoporosis. Originally, he was going to start tapering right after the rtx (and put on Imuran) and cut 5mg per month, but didn't do this and wanted her done with pred after month 6. She couldn't tolerate the Imuran, so now, she is on Mofetil.

[drz]

The dipstick urine tests are just another monitoring device to increase chances of early detection. If I should get an abnormal reading on one I would get a regular full lab work of blood analysis and urinalysis right away to verify if their is really a problem. Otherwise I would just get my regular monthly labs.

[mrtmeo]

Update:
Talked with a rheumy that consults with Carol Langford and he was very surprised that my mom did not respond well to the rituximab.
He says that the current treatments he uses is 2 infusions, 2 weeks apart instead of the 4 infusions, 1 week apart.
I really think the reason my mom has not responded well is due to her tapering off a benzodiazepine which probably triggered (cytokine storms) the vasculitis.

I sure hope the mofetil works.
It looks like the Rtx will end up the treatment of choice.

[annekat]

I'm just so glad you are now with a rheumy who sounds like he knows vasculitis and its treatment and who consults with one of the best. I hope your mom will show great improvement now under his care.

[mrtmeo]

I'm just so glad you are now with a rheumy who sounds like he knows vasculitis and its treatment and who consults with one of the best. I hope your mom will show great improvement now under his care.

Thanks Anne.
At this point, I have lost all hope.
Her proteinuria only showed improvement the week after starting Losartan, but went up again a week later.
One Nephrologist said that the kidneys can go into auto destruct mode until they completely fail.

Looking at dialysis doesn't show much hope either when the proteinuria is high because it keeps going up and nutrition goes way down.
I can't give her my kidney unless she goes into remission and passes a lung function test for surgery.

When I asked the rheumy if the mofetil would correct the proteinuria, he said that proteinuria is very tricky.
I took it as, it won't help.
I don't understand the mechanism that causes the proteinuria.

I tried adding BCAA's (Branch Chained Amino Acids) to her diet and it greatly increased her albumin, hemoglobin and hemocrit.
However, her proteinuria is worse which I think is kidney hyperfiltration and may reverse now that I stopped the BCAA's.

[annekat]

Blake, I'm so sorry you have come to the point of feeling so hopeless. I wish the new rheumy could offer more encouragement. I think you did say that more RTX appeared to be indicated, and I'll be interested to hear how that goes if it happens. But I do understand the kidneys can reach a point of no return. You know much more about the technical aspects of all this, and unfortunately I don't currently have time to research some of the terminology you used. I can only hope that things aren't as bad as they seem and there is some chance that they will improve. Did the rheumy say what the next step would be and when?

[mrtmeo]

Blake, I'm so sorry you have come to the point of feeling so hopeless. I wish the new rheumy could offer more encouragement. I think you did say that more RTX appeared to be indicated, and I'll be interested to hear how that goes if it happens. But I do understand the kidneys can reach a point of no return. You know much more about the technical aspects of all this, and unfortunately I don't currently have time to research some of the terminology you used. I can only hope that things aren't as bad as they seem and there is some chance that they will improve. Did the rheumy say what the next step would be and when?

Hi Anne,
I suppose there is always hope, but I am so discouraged by my mom's lack of response to treatments and the failing in some of her medical care.
There is the possibility that her kidneys could heal to a point that is better, but there is always a flare that can drop the function quickly.
I was hoping her kidney function would have gone at least to 3rd stage, so that she has a little chance if a flare comes along again.
If the protein dumping doesn't resolve, she will probably end up in end stage renal failure anyway.

If you are wondering what hyperfiltration is, it is when normal kidneys will dump excess protein due to too high a protein diet.
This is a way the kidneys adapt and doesn't harm the kidneys.
The kidneys will go back to zero protein when the person lowers their protein intake.
This happens alot with body builders.

[mrtmeo]

For MPA, I am beginning to think that doing at least 2 months cytoxan and then a maintenance seems more effective.
My mom's kidney function responded to the single boulus dose of cytoxan she got 2 weeks before the rituxan.
Once she got on the rituxan, she went into a big flare that lasted months.
Perhaps, rituxan for maintenance, but I still have not found a single MPA patient that went into remission using rituxan first.

For weg's, rituxan looks very favorable for induction of remission and even better for maintenance.
Just my thoughts.

[annekat]

Hmm, maybe it's time for another round of both CTX and RTX for your mom. I think I've heard that even for GPA patients, RTX doesn't always work well the first time. For others, I know it works great, though it may take a few months. I guess you have another appt. with her rheumy before too long and it will be interesting to hear what he thinks of all this. I hope she is still feeling a little better these days than she was for awhile.

[mrtmeo]

I will discuss a round of ctx with the rheumy, but not sure if he would do it.
Her lymphocytes are climbing, so I think the rtx is wearing off.
The rtx never really improved the creatinine except for .1.
2 weeks after the one ctx treatment and a blood transfusion her creatinine went from 3.3 down to 1.9.
After this, she was put on rtx and her creatinine went up and down but never below 1.8.
On the mofetil, she is down to 1.8, so far and the anemia seems to have some improvement.
She is also getting acupuncture and her acupuncture dr said that anemia can go up and down until resolving.
Getting the right treatment is a crap shoot.
It sure seems common that most people go into remission within the 2nd -3rd month of treatment, but alot of damage can happen during this time if they don't.

She needs another anca test to see if she is negative, yet.
At least, if she was negative, I would know she was healing.

[annekat]

It would be nice to see a negative ANCA, for sure. My ANCA was barely positive at dx and I haven't had another test. Don't think I need one at this point, because I have almost no symptoms and a normal SED rate, and have always had normal creatinine. But if your mom has always had an elevated ANCA since first tested, I agree you'd want a update to see if there's any improvement. I wonder if some CTX would help the kidneys more than the RTX has. I hope your rheumy is open to that idea. If not, I hope that another round of RTX will make a difference. Lots of wondering and hoping going on.... best wishes to you and her. I know this has been a very hard road for you both to travel on.

[mrtmeo]

It would be nice to see a negative ANCA, for sure. My ANCA was barely positive at dx and I haven't had another test. Don't think I need one at this point, because I have almost no symptoms and a normal SED rate, and have always had normal creatinine. But if your mom has always had an elevated ANCA since first tested, I agree you'd want a update to see if there's any improvement. I wonder if some CTX would help the kidneys more than the RTX has. I hope your rheumy is open to that idea. If not, I hope that another round of RTX will make a difference. Lots of wondering and hoping going on.... best wishes to you and her. I know this has been a very hard road for you both to travel on.

I don't know if being 7 months from first infusion if she could still get improvement with another treatment.
Do you haven any fatigue?

[annekat]

I don't know if being 7 months from first infusion if she could still get improvement with another treatment.
Do you haven any fatigue? I don't know either. People with RTX experience could possibly answer that.

Yes, I still get fatigued a little easier than I did pre-Wegs. But it is vastly improved. It's partly due to permanent lung damage, which in my case is relatively minor I think, but is still there, so I get out of breath a little sooner. I also get the mental fatigue, which could be partly due to the meds, or maybe some of my brain cells have been destroyed, although if so, not to a great degree. The fatigue seems to be ongoing for most Weggies, even those in remission. But I'm doing well. I just took a mile long walk for the third day in a row, and although I know that is not a great distance, I have noticed more stamina each day, for sure. So some of the fatigue on the first day was just being out of shape. I'll be increasing the length of the walk soon.

[mrtmeo]

I don't know either. People with RTX experience could possibly answer that.

Yes, I still get fatigued a little easier than I did pre-Wegs. But it is vastly improved. It's partly due to permanent lung damage, which in my case is relatively minor I think, but is still there, so I get out of breath a little sooner. I also get the mental fatigue, which could be partly due to the meds, or maybe some of my brain cells have been destroyed, although if so, not to a great degree. The fatigue seems to be ongoing for most Weggies, even those in remission. But I'm doing well. I just took a mile long walk for the third day in a row, and although I know that is not a great distance, I have noticed more stamina each day, for sure. So some of the fatigue on the first day was just being out of shape. I'll be increasing the length of the walk soon.

It kinda sound like your fatigue is really just endurance.
The pred has really done this to my mom.
However, when she had leukopenia for a couple days, she energy to do stuff, but that all changed when her neutrophils went sky high again.
Her neutrophils are always above normal even when the rtx kept the lymphocytes below .2.
I really think the vasculitis causes damage to ATP which is our energy source and remember reading about it somewhere.
I just can't remember where I read it.

I am glad that the more you walk, the better you feel.
That is encouraging.

[annekat]

There are different kinds of fatigue. With the walking, I think I'm just like anyone else at this point and if I don't do it for awhile, it will take at least a few days to build up the endurance, so I agree with you there. The other kinds of fatigue for me that may be more WG related are like having to engage in an activity with people for a few hours and needing to get home and rest. That is more like a mental fatigue combined with whatever physical activity, or having a commitment to be somewhere and "on" for a period of time, is involved. I have always tended to need down time, and with WG, I think this becomes more so.

[mrtmeo]

There are different kinds of fatigue. With the walking, I think I'm just like anyone else at this point and if I don't do it for awhile, it will take at least a few days to build up the endurance, so I agree with you there. The other kinds of fatigue for me that may be more WG related are like having to engage in an activity with people for a few hours and needing to get home and rest. That is more like a mental fatigue combined with whatever physical activity, or having a commitment to be somewhere and "on" for a period of time, is involved. I have always tended to need down time, and with WG, I think this becomes more so.

Would you say that the fatigue you get when being out with people is all mental fatigue or is there some physical fatigue with it?

[annekat]

Would you say that the fatigue you get when being out with people is all mental fatigue or is there some physical fatigue with it? It is probably some of both. It can be physically as well as mentally demanding to be in a social situation where you are committed and can't leave. You may have to be on your feet for a long time or sit in uncomfortable positions, or you may be working together with others to move things around or accomplish some other physical task. When I'm working by myself at home, I can rest or take a break whenever I want, and that is not always true in social situations. Sometimes just shopping in a grocery store or doing a string of errands can wear me out, and I'll cut my list short so I can go home.

[mrtmeo]

It is probably some of both. It can be physically as well as mentally demanding to be in a social situation where you are committed and can't leave. You may have to be on your feet for a long time or sit in uncomfortable positions, or you may be working together with others to move things around or accomplish some other physical task. When I'm working by myself at home, I can rest or take a break whenever I want, and that is not always true in social situations. Sometimes just shopping in a grocery store or doing a string of errands can wear me out, and I'll cut my list short so I can go home.

oh, that really sucks.
I am so sorry that most vasculitis folks suffer that kind of fatigue all the time.
It tells me there is still some kind of smoldering disease process going on killing your energy.
I sure hope they come up with a drug that can stop the smoldering.
Have u ever heard from long term weg folks if they get their energy back after many years?

[annekat]

oh, that really sucks.
I am so sorry that most vasculitis folks suffer that kind of fatigue all the time.
It tells me there is still some kind of smoldering disease process going on killing your energy.
I sure hope they come up with a drug that can stop the smoldering.
Have u ever heard from long term weg folks if they get their energy back after many years? Actually, my fatigue level has gotten a lot better over the last year or so. What I was describing was more typical of an earlier time period. But I still have to be careful, it can take me by surprise. I don't think there is anything much smoldering in me right now, as my inflammation level is completely normal according to my last blood work, and my symptoms have settled way down. I'm still on meds. I could have another flare this winter but so far don't feel that way. I think many Weggies still report some issues with fatigue indefinitely, but there are other factors such as age, as well. We have some people on the forum who seem to manage whatever fatigue they have pretty well; Pete in Ohio, Don in Arizona, and Marta in Alberta, Canada, are three who come to mind and I'm sure I can think of others. I don't know that they have no fatigue, but they seem to lead pretty active lives including physical activities that they enjoy.

[mrtmeo]

Renal Outcomes of Cytoxan:

Looking at this study shows a more realistic result of renal improvements with cytoxan treatments.
Clinical features and outcomes of ANCA-associated renal vasculitis Seck SM, Dussol B, Brunet P, Burtey S - Saudi J Kidney Dis Transpl (http://www.sjkdt.org/text.asp?2012/23/2/301/93162)

As far as Weg's, It looks like cytoxan for 2 months and rituxan for maintenance maybe starting around the 4th month would work ideal.
Any other thoughts out there?
Of course, this is just theory.

So far, with MPA, it looks like 2-3 months of cytoxan followed by Imuran for maintenance works very well.

Rituxan didn't improve my mom's kidney function with MPA.
She had 1 week iv dose of cytoxan along with high dose salumedrol and her creatinine went from 3.3 to 1.9 in two weeks.
I sure wish I would have stuck with the cytoxan.

This kid with MPA had a creatinine of 9 at his worst and 4.5 years later, his creatinine is 1.2 using cytoxan and imuran.
https://www.youtube.com/watch?v=zkxkq5QjOhc&list=PL451A815B784B2048&index=12

[annekat]

As far as Weg's, It looks like cytoxan for 2 months and rituxan for maintenance maybe starting around the 4th month would work ideal.
Any other thoughts out there?
Of course, this is just theory.

I haven't taken the time yet to look at the links you posted, but your theory above sounds interesting to me. It would be a good thing to be on CTX for less time but give it a chance to zap the worst of the symptoms before switching to RTX. However, I'm feeling like it might be better to either stay on the CTX a little longer or start the RTX a little sooner and not have a gap with no treatment between the end of the 2nd month and the beginning of the 4th month, if that is what you meant. Or the CTX and the RTX could overlap for a time, unless someone knows of a reason they shouldn't. I just don't think most people go into remission after 2 months of CTX, though I'm also not sure if that's what you meant. These are just my gut feelings, and of course, everything would depend on how the patient was responding to the treatment. It appears there are many creative and innovative treatment schedules that can be followed with the WG meds, and I like seeing that, as long as the docs are experienced and know what they are doing. Would be interesting to know how a WG specialist would respond to your theory.

[mrtmeo]

I haven't taken the time yet to look at the links you posted, but your theory above sounds interesting to me. It would be a good thing to be on CTX for less time but give it a chance to zap the worst of the symptoms before switching to RTX. However, I'm feeling like it might be better to either stay on the CTX a little longer or start the RTX a little sooner and not have a gap with no treatment between the end of the 2nd month and the beginning of the 4th month, if that is what you meant. Or the CTX and the RTX could overlap for a time, unless someone knows of a reason they shouldn't. I just don't think most people go into remission after 2 months of CTX, though I'm also not sure if that's what you meant. These are just my gut feelings, and of course, everything would depend on how the patient was responding to the treatment. It appears there are many creative and innovative treatment schedules that can be followed with the WG meds, and I like seeing that, as long as the docs are experienced and know what they are doing. Would be interesting to know how a WG specialist would respond to your theory.

Hi Anne,
I should have mentioned that I meant a minimum of 2 months cytoxan.
Some folks with weg's don't seem to respond to cytoxan and lose their kidney function, so if they haven't had any response from cytoxan on their organ function after 2 - 3 months, they could move to rituxan.
It seems most people go into remission around the 2nd and 3rd month from treatment, but those that don't respond can have further organ damage and need an alternative.
I wish they would come up with a time frame that could tell them if it is time to try another drug to get the person in remission to minimize damage.

Maybe when the research is out with the plasma exchange, they could use that for people with lung kidney issues to stop the ANCA's while their drug is working?
Right now, they won't use plasma exchange unless the creatinine is >5.6 or the lungs are life threatening.
I think the trial is plexvas, but not sure.

[annekat]

I still think remission is a subjective and somewhat loosely used word. I was not in what would probably be called remission after 3 months of CTX, but I had shown great improvement, so obviously the drug was working. I was kept on it several months longer than that, but think I could have been switched to MTX sooner, as my lungs were doing well and I had no kidney involvement. I don't think I needed RTX. But for someone with more severe and harder to treat issues, I agree that starting with CTX and then switching to RTX could be a good route to take, even if they had shown some improvement with the CTX, the point being to limit the use of CTX in one's lifetime, with it's known dangers with long term use. The CTX would kick-start the process and then the RTX would take over. True, some may not respond to CTX at all, but I think they are rare, from my readings on here.

[mrtmeo]

Valid points.
I believe that if there are high inflammatory markers and pos anca, the disease is smoldering somewhere.
Of course, there are some that are not anca pos, but they would still have high inflammatory markers with active disease.

[annekat]

Valid points.
I believe that if there are high inflammatory markers and pos anca, the disease is smoldering somewhere.
Of course, there are some that are not anca pos, but they would still have high inflammatory markers with active disease. Yep. I have no idea what my ANCA is, because it never seems to get tested and was barely positive at dx, although I was quite sick. But I know from my last blood work that my inflammatory markers are now normal! So I guess that means there is no active disease at present. Maybe someone would say I'm in remission, though no one has used that word, and it would definitely be a medicated remission. Maybe if I switched docs I would finally hear the R word!