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View Full Version : Weg flare without any detectible anca markerrs possible?



usrgbt
10-14-2013, 10:07 AM
Diagnosed with WG about five years ago, affecting primarily my lungs and causing moderate scarring and interstitial lung disease. On 2 lt O2 at night. Have been doing fine and in remission until about 3 months ago when my energy level suddenly plummeted and I desaturated precipitously upon standing. Blood test which I had been taking regularly since being diagnosed revealed that my white and red blood cells as well as other blood results had declined significantly out of acceptable range. Anca markers were non detectable. After some delay, I was finally admitted to the hospital at which time I was diagnosed with pneumocystic pneumonia. In addition, they diagnosed me with myelo dysplasia. Because of the non detectable anca markers, my rheumatologist does not believe the Wegner's is flaring. My question is is it possible for Wegners to be active without anca markers? I am currently being treated with rituxan, 80mg prednisone, and vidaza for the dysplasia. Am on 4 lt O2 at rest and 6 lt with activity, but still rapidly desaturate rapidly upon standing or movement. Anyone heard of anything like this combination of symptoms or have any suggestions. Anything is appreciated. Never been so out of gas in my life!

Thanks,
Gary
Lake Saint Louis, MO

Dirty Don
10-14-2013, 11:25 AM
Just finished a mini flare...docs upped/restarted pred, it worked. Not sure you'd want/need that at 80. But I did have a fairly quick lapse into lethargy and stomach issues. None of my markers were out of sight or terribly suspicious. My sed rate was a bit high, nothing else. I responded quickly to a dose change. Best of luck.

usrgbt
10-14-2013, 01:12 PM
Thanks Don, I appreciate the info. Glad you were able to get on top of your flare quickly and successfully! Still working with my issues, but felt a little stronger today. Now if I can keep it up everyday! Anyway, thanks again!

annekat
10-14-2013, 03:23 PM
I've read on here many times that not everyone with Wegs, or who is having a flare, has much of an ANCA reading at all, so it is not a reliable indicator. I had such a low reading at onset that my doc, not a Wegs specialist by any means, did not think I had it, although it had been suggested by another doc that I did, given the results of a CT scan of my lungs. It was confirmed by a nasal biopsy. As for flaring, inflammation markers would be more of an indicator, along with increase in symptoms.... however, the fact that you had pneumocystic pneumonia would complicate this issue, as that also would cause a rise in these markers, I presume. Were you taking Bactrim, or other brand name for the sulfa antibiotic that a lot of us take regularly to prevent this pneumonia? Just curious. I just recently was unable to get my Bactrim for about a week and was kind of scared... also I am attempting to taper pred at the lower level doses... I started coughing quite a bit more, but am now thinking this was more likely to be from the pred taper than from a few days of missing Bactrim. So anyway, you might or might not be flaring, but I think your doc is wrong to write off a flare just because of a low ANCA reading. I'm sorry about the fatigue... we have certainly all been through that and still are from time to time.... but it also seems like having pneumonia and the other things you mentioned would take a lot from your energy level. I hope things start to get back to a more normal level soon!

usrgbt
10-14-2013, 04:15 PM
Thanks so much for the information Annekat. I have not been on bactrim since my original bout with Wegener's when I was on it for about a year. Is it something I should discuss with my doc about taking it regularly? I suspected the lack of anca markers didn't preclude a flare. My inflammation markers were very high. Lungs have taken a beating. PFT reveal about 30% loss from last pre illness test. Anyway, thanks again, and any I'm grateful for any ideas, suggestions, or things I should talk to my doc about.

annekat
10-14-2013, 06:36 PM
I am certainly no expert, far from it, but yes, since you are on immunosuppressants, it seems to me you should talk to your doc about taking Bactrim, especially since you recently had the pneumonia that it is taken to prevent. I'm not clear on whether you were on the RTX or other immunosuppressant at the time you got the pneumonia. But the reason most of us take the Bactrim is because we are on the immunosuppressants, whether it be CTX, MTX, RTX, AZA, or others, along with pred, and that therefore our immune systems are less likely to be able to fight off this particular type of pneumonia that people with healthy immune systems can normally fight off, or so I've heard. Also, people with AIDS commonly get this pneumonia, and it can be deadly, because while they are not on the immunosuppressants like we are, their immune systems are severely deficient as a result of their disease. I'm kind of figuring that, though in remission, you were still on an immunosuppressant when you got the pneumonia. I could be wrong, of course, and you know better than I, but I have heard this particular infection is not a problem for people whose immune systems are not suppressed or deficient. Then again, a person with Wegs has an improperly functioning immune system, so I wonder if even if not being suppressed by drugs, it can still fail to fight off certain infections because it is so busy attacking parts of our bodies it is not supposed to. That is purely speculation on my part, and no doubt someone here knows way more than I do.

As for the lungs and PFT results, I know from reading on here that a lot of us with lung involvement have permanent damage and while we may be doing well, may never have the lung function we did pre-Wegs. But if yours has recently worsened from what it was during your definite period of remission, and before your recent downturn, then yes, I'd want to be sure there is not Wegs in your lungs again.... however, the bout with pneumonia could also be responsible for that, I'd presume. It sure gets tricky when Wegs coexists with other conditions and we can't be sure which is causing what symptoms.

usrgbt
10-15-2013, 12:16 AM
I can't begin to thank you for all the information you have shared. I've learned more helpful things to talk to my Rheumatoligist than since I developed Weg's! You are correct that I was on Immuran (Azathioprine) after initial treatment with Cytoxan until the recent decline in blood test results. Dr. thought for sure the Immuran caused the decline in white and red blood cells and immediately had me stop taking it, but it had no effect. As I mentioned my rheumatologist and a hematologist have concluded that I have developed myleoplasic Dysplasia (pre leukemia)) which is responsible for the blood test declines. They are treating it with three rounds of daily Vadaza infusions for three weeks, with a week off between sessions. I'm also getting once weekly infusions of Rituxin for four weeks. Plus my pulmonologist has me on 80 mg prednisone and sulfameth/trimethoprim 800/160 mg antibiotic. Regarding the lungs, he basically confirms the damage is pretty much permanent. Something does seems to be working thankfully, because for the first time in weeks, I feel a bit stronger. Anything is an improvement! Anyway, just getting ready to head for the infusion center for Vadaza treatment and also possible red blood cell transfusion, which they also have given me three times,plus one transfusion of platelets. Thank you so much for your kindness and helpful information. I truly appreciate it!

Gary

Debbie C
10-15-2013, 01:56 AM
Hi Gary, you are definately being given enough drugs !!!! When I was first dxed with wg in my lungs also, my anca tests were neg for months. The only way they knew it was wg was thru a lung biopsy. My dr. at Cleveland Clinic doesn't even check my ance at all. He said the best thing to look for is sed rate incline. Hope you start feeling better soon and they can cut back on some of your drugs. The rtx infusions should really do it. Take care and keep us posted

annekat
10-15-2013, 04:04 AM
Yes, the drug that starts with "sulfa" is the generic name for the brand name Bactrim, so yes, you are taking it. That is good. I also take the generic version, but Bactrim is a lot easier to say. It sounds like your docs have things pretty well nailed down.

usrgbt
10-15-2013, 09:17 AM
Thanks Deb, for your information. It seems clear that relying on anca markers to determine Wegener's activity is not reliable and should only be considered one part of the puzzle. I'll be talking with my rheumatologist about it. I appreciate it!

usrgbt
10-15-2013, 09:20 AM
Thanks, Anne!

Alysia
10-15-2013, 11:58 PM
I just found this :
Performance evaluation of three assays for the detection of PR3-ANCA in granulomatosis with polyangiitis in daily practice (http://www.sciencedirect.com/science/article/pii/S1568997213001134)

drz
10-16-2013, 09:21 AM
Thanks so much for the information Annekat. I have not been on bactrim since my original bout with Wegener's when I was on it for about a year. Is it something I should discuss with my doc about taking it regularly? I suspected the lack of anca markers didn't preclude a flare. My inflammation markers were very high. Lungs have taken a beating. PFT reveal about 30% loss from last pre illness test. Anyway, thanks again, and any I'm grateful for any ideas, suggestions, or things I should talk to my doc about.

What is total loss of pulmonary function compare to normals your age?

Is pulmonary rehab an option for you. I found it very helpful for me.

drz
10-16-2013, 09:23 AM
I just found this :
Performance evaluation of three assays for the detection of PR3-ANCA in granulomatosis with polyangiitis in daily practice (http://www.sciencedirect.com/science/article/pii/S1568997213001134)

Thanks for sharing this reference.

usrgbt
10-16-2013, 10:16 AM
I'm not sure what normal pulmonary loss for my age (71) is. All I know is that I'm on full time oxygen at 4 liters at rest, and 6 liters w/activity as a result of the recent illness. Prior to the recent situation, I was only on oxygen at night at 2 liters. I'm hopeful I can regain a little function with continued treatment. I have had pulmonary rehab and it did help, but probably not able to do right now because of weakness.

usrgbt
10-16-2013, 10:17 AM
Thanks for sharing!

Tom
11-27-2013, 02:35 AM
Diagnosed with WG about five years ago, affecting primarily my lungs and causing moderate scarring and interstitial lung disease. On 2 lt O2 at night. Have been doing fine and in remission until about 3 months ago when my energy level suddenly plummeted and I desaturated precipitously upon standing. Blood test which I had been taking regularly since being diagnosed revealed that my white and red blood cells as well as other blood results had declined significantly out of acceptable range. Anca markers were non detectable. After some delay, I was finally admitted to the hospital at which time I was diagnosed with pneumocystic pneumonia. In addition, they diagnosed me with myelo dysplasia. Because of the non detectable anca markers, my rheumatologist does not believe the Wegner's is flaring. My question is is it possible for Wegners to be active without anca markers? I am currently being treated with rituxan, 80mg prednisone, and vidaza for the dysplasia. Am on 4 lt O2 at rest and 6 lt with activity, but still rapidly desaturate rapidly upon standing or movement. Anyone heard of anything like this combination of symptoms or have any suggestions. Anything is appreciated. Never been so out of gas in my life!

Thanks,
Gary
Lake Saint Louis, MOI wish I could answer that question but early on in my journey my RA told me that there is no blood test or lab draw that can say if you are having a relapse of the other way, in remission. He said that as long as I feel well and now symptoms and the blod work looks as normal as it could, I would be considered in remission. He admits that it may not be the case and that is why I am monitored regularly and I need to listen to my body. Just like when I went with a full blown flare up after fighting symptoms for a couple years, the purpura was a big clue and then reading my prior history with sinus and joint pain and swelling. That only warranted a biopsy. I had a lobectomy 2 months before because of a growth t hat wasthoght to e cancer because I have a history with cancer but turned out to be what was called at the time a hematuria tumor or something like that which is benign. The purpura led them to a biopsy of the kidneys and then had to wait for lab results although the RA was certain of Wegs but had to wait for confirmation and my treatment was hard because I had stage 4 colon caner in 2006 and some of the meds for treatment was used for cancer treatment also and I could only have so much of it in a lifetime. But anyway, personally I would say you are having a flare up and should see the Doc. I am on 2 liters of 02 and was told I could go to 3 liters but resisting right now. Good luck to ya.

D.B.
11-27-2013, 03:54 AM
Thanks Deb, for your information. It seems clear that relying on anca markers to determine Wegener's activity is not reliable and should only be considered one part of the puzzle. I'll be talking with my rheumatologist about it. I appreciate it!
hi gary hope u r feeling ok. yup this disease is quite a puzzle. as patienta we need to b in tune w our bodies to notice changes that arent quantifiable by lab work. how long have u been @ 80 mg. did dr mention when start tapering? 60 mg makes me so irratic and hungry but also gets my energy level back up.

usrgbt
11-27-2013, 03:17 PM
Thanks for the information, Tom. I appreciate it. I don't think my RA is convinced I had a flare, but I'm pretty sure I did. The pneumonia and Mylea Dysplasia has complicated things, but I am slowly recovering and have gained quite a lot of my strength back. The prednisone really got to my legs and they are still pretty weak. I'm down to 40mg daily pred. Lungs took a beating and I'm on 4 at rest and 6 with activity, but it's really not enough.Desaturate when I get up,but recover fairly quickly when I sit back down. Anyway,it is what it is. Hopefully I can recover a bit more.

usrgbt
11-27-2013, 03:26 PM
I know what you mean about the pred. I'm down to 40 mg daily, but still want to eat everthing I can get my hands on. The other problem is it makes me so shaky I can barely feed myself sometimes. It also took a toll on my strength, but have started recovering some with the reduction of the pred.

Tom
11-27-2013, 04:06 PM
Eating is one problem. I was a grazer before all of this happened. the worst part was the roid rage. I became aware of the rage when I started cancer treatment in 2006 and in the first week, I wanted to drop the pastor while in church because he said good morning and blessings and thanks for coming. I wanted to hit anyone who got near me. When asked by my oncologist how I did the first week of my treatment I said I was doing well, but the wife said he has been exceptionally mean and aggravated. I denied it in a very terse way and the oncologist immediately lowered the amount of steroid I was going to get. MAN, I'm glad I told my wife how I was feeling. I was mad as hell that they accused me of being enraged! On the pred., I still wanted to eat anything in sight but when they lower my pred and then have to raise it again, I get aggravated but am now aware of the rage and can control it. I am so ashamed, I wanted to take out the pastor for crying out loud. He is a regular friend outside of church but I have never told him but will some day.

usrgbt
11-28-2013, 01:07 AM
I've experienced all of the exact things you mentioned,including the emotional ups and downs. Thank you for posting. I've tried to explain it to my wife but I let her read what you experienced and she has a better understanding now. Pred is definitely a double edged sword!

D.B.
11-28-2013, 01:19 AM
Eating is one problem. I was a grazer before all of this happened. the worst part was the roid rage. I became aware of the rage when I started cancer treatment in 2006 and in the first week, I wanted to drop the pastor while in church because he said good morning and blessings and thanks for coming. I wanted to hit anyone who got near me. When asked by my oncologist how I did the first week of my treatment I said I was doing well, but the wife said he has been exceptionally mean and aggravated. I denied it in a very terse way and the oncologist immediately lowered the amount of steroid I was going to get. MAN, I'm glad I told my wife how I was feeling. I was mad as hell that they accused me of being enraged! On the pred., I still wanted to eat anything in sight but when they lower my pred and then have to raise it again, I get aggravated but am now aware of the rage and can control it. I am so ashamed, I wanted to take out the pastor for crying out loud. He is a regular friend outside of church but I have never told him but will some day.

dont be so hard on yourself. i, too, experience that rsge. noone can understsnd unless they have been through it. your body is suddenly not your own and that csn be hard to deal with. my BF kinda gets it now and calls me miss prednisone when i get "irratic" and mean (which is nothing like my drug free self)

Tom
11-28-2013, 08:16 AM
DB thanks for the support and the understanding. Im past it now of course but I had on event with a truck driver who tooted his horn at me at red light because I wouldn't turn right on the red as I was going to pulmonary rehab. It just so happened that he was going to pick up a piece of equipment at the hospital construction site. I walked across the street and promptly told him that if he blows that horn at the wrong person they shove it you now where! There was a 10ft chainlink fence between us and he spouted some foul language and told his co worker that the dumb S.O.B. just sat at the red light and he could hae gone on it. I informed him that the law I may if the way is clear and not shall or will and I can sit the all day if I want and the light is red. I told him that fence and do the horn thing myself. I finally told myself that I have to walk away from this as there I stood with my o2 tank over my shoulder So that's just what I did. A couple days later I was telling my brother inlaw about it and he said his buddy Joe drives for that construction company and he was the only driver. So Joe got razzed by the BIL and when Joe found out who I was and was told my story of medical problems, he laughed and said he needs to get me a company T-SHIRT. The BIL said they laughed and when he told me how Joe reacted, it became funny. He said Je and I will hit it off good because he has a personality like mine where neither will stand down but will respect each others space. So we will get together sometime and laugh about it Joe and I. But your right, unless you have not experienced, you cant be told to relax or calm down with out getting irritated. But like I said, I recognize it now and I know how to deal with it. I think there is a bit of personality traits that can determine your level of tolerance with the steroid. I was bullied as a kid and early in life I was protected by other bigger kids and in some cases bullies themselves. About the 3rd grade I became the guy who bullied the bullies and looked after other bullied kid and in particular 2 severe downs syndrome kids. I found, or the bullies found that I had a mean streak and short fuse to go with it they left alone and those I stood by. One of the controls I found if I was on a pred increase was if I felt a little edgy I would tell my wife and then head out to the garage and read and build something or she would treat me with kid gloves. She is so understanding.

Happy Camper
11-28-2013, 08:59 AM
I'm currently having my 1st flare up. MY ANCA is negative. Its never even been positive. My diagnoses was confirmed by open lung biopsy. My sed rate was a little higher then normal 18. I have nodules on both lungs, again. Hard to know when its active when my blood work basically remains the same. I didn't feel "normal" but I didn't really feel that sick either. LIttle burning of the eyes, lung pains, etc... Not sure how I'm going to tell when a flare is coming in the future. So frustrating!