Weg flare without any detectible anca markerrs possible?

[usrgbt]

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]

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]

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]

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]

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]

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]

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]

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]

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]

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!