Im new and Ineed some information on p panca postive and MPO positive

[maria garcia]

I have so many questions and a bad attitude doctors who is no help. My daughter was diagnosed with ppanca poistive and mpo positive she is only seventeen. I had never heard of this auto immune disorder. My doctor doesnt not want to explain anything except treat her for a eye condition which we fell is associated with the disease we will know tom. In two weeks he said we will sit an talk about. This all started with a red eye send us to a rheum, and my whole life has changed forever! Im so lost and new in this. Maybe you can help. All her other blood work came out fine but there doing a chest xray and a sinus. I think they might me suspecting Wegener's even though he c anca was not high normal range but she has had the sinus symptoms, bleeding of the nose and alot of dry cough. I was always told is allergies and im praying it is. I have been reading alot in the Internet and im overloaded with information and depression. I even read her life span can be two years. I had a mental breakdown. I need to be strong for her and im not sure how. Please explain to me a little about this disease and how the number work. And Im also trying to figure out the difference between the autoimmune disorders and why this one seems so much worse. I will appreciate any information. thank you for your time. ines

[pberggren1]

I'm so sorry to hear about your daughter Maria.

Wegener's can present in many areas but the sinuses and nose are very common.

There is a thread on here called: What the newly diagnosed need to know. Try reading through that thread.

Also go to the vasculitisfoundation.org It will have a bunch of info on Wegener's.

But for the most part Wegs can affect the joints with joint pain that can come and go, sinus headaches, post nasal drip, blocked nasal passages, crusting and bleeding in nasal passages, coughing up of blood and/or mucus, trouble breathing, loss of appetite and weight, hearing loss, loss of vision, sores in mouth, skin rashes, neuropathy, and the list goes on and on.

Diagnosis can be tough. Usually a biopsy is prefered of the nose, lungs, or kidneys if they are suspect of being involved. But a biopsy can come back negative and it could still be Wegs.

Also ANCA is a very unreliable test to determine Wegs. In some people it is a reliable marker but for most it is not. I have had Wegs for over 8 years now and ANCA was a reliable marker for me until just recently. I am C-ANCA positive but sometimes the P-ANCA creeps up as well.

Other blood tests to look for are ESR or sed rate, CRP, Creatinine, WBC.

I hope this helps somewhat. I am not a parent so I can not relate directly to what you are going through but my parents have basically been looking after me the last few months because of a flare up and a bad lung infection.

[maria garcia]

Thank you so much for taking the time to answer. I have beena mental mess. Having a doctor who doesn't answer questions. Told me to look in the inernet and I almost died when Iread 2 years of life if not treated. I see you have been coping with this for several years good for you. My daughter started with a red eye.And if the conclusion is vasculitis then he mentioned chemo and cortisone. I feel like noone I talk to knows about this disease. she is so young Iwould give my life to suffer instead of hers Iwish it would work that way.

[maria garcia]

What are considered high number for p Anca ? My daughter has is having another blood work done and she has been drinking antibiotics do you think it can effect the results. Ive hd mixed answers.

[pberggren1]

Where do you guys live Maria? The main Vasculitis Centers are: Mayo Clinic in Rochester, MN, Cleveland Clinic in Cleveland, OH, Boston University in Boston, MA, Johns Hopkins University in Baltimore, MD, and there is one in California somewhere and one in Salt Lake City, Utah. I think there is also one in Durham or Chapel Hill area.

What I am getting at is that if it is Wegener's one has to make a big effort to get to one of these places or at least have a better doc that is will to consult with one of the Wegs Specialists at one of these centers. And if you can right now go to one of these centers now for diagnosis.

[pberggren1]

What are considered high number for p Anca ? My daughter has is having another blood work done and she has been drinking antibiotics do you think it can effect the results. Ive hd mixed answers.

I'm not sure if antibiotics will affect blood tests. But if she just started them I would think not. But maybe it could affect the White blood count or ESR or CRP, but I am just guessing here.

ANCA results are given two ways: As a titer number like 1:40 or a just a straight number like 5. Mine are always just given as a straight number. So I think that anything over 6 to 8 and higher would be positive and a 10 would definitely be positive. Mine in the past have been as high as 40. But I do not know what a high or positive titer would be.

[JanW]

Maria -- I think it would help us to know where you are located so that we could direct you to a vasculitis center. Even if you cannot travel to one, the experts can consult with your doctor free of charge.

There is some good information on the internet about WG, but bad information as well. For instance, many of us went years undiagnosed (I think about five in my case) and we didn't die. But the important part is to get treated. You should go to the Vasculitis Foundation's website and see what they say about p-ANCA. It is often associated with Churg-Strauss syndrome, which is probably the second most common vasculitis (the first being WG, but they are all rare). Don't worry, many of the vasculitis conditions are treated the same -- with steroids and a chemotherapeutic agent such as methotrexate or cytoxin.

It sounds to me like you really need answers and you need them quickly. I'm sure others will be along this morning with their thoughts. Hang in there because vasculitis is treatable, and your daughter can live a long and productive life with it.

[NicShaf]

Maria,
I'm sorry to hear about your daughter, she is so young to be afflicted with this disease. However, like Jan and Phil mentioned above, Wegs is treatable, as long as you get your daughter treatment as soon as possible, she has a very good chance of recovering, especially since she is so young. I have about 10 years on your daughter, I was 26 at diagnosis, but my doctors reassured me that my age was on my side and my body could take the treatment well and recover.
It would be helpful to have a doctor who answered your questions, we had a similar Rheumy in the beginning, and it made a huge difference to get someone who would listen and answer any questions me and my family had. I hope you can either find a better doctor or have your doctor consult with a Wegs specialist at one of the centers.
I have C-ANCA, and I know that any ANCA count with this is abnormal. I'm not exactly sure if it's the same for P-ANCA. Normal is considered below 1:40. When I was hospitalized in December, mine was 1:360. It went down when I started steroids to about 1:120, and now is at 1:40 after about 4 months of Cytoxin.
On another note, you will read on this site about many who have achieved remission and maintained it for many years, some 10-20 year spans. There is no reason to fear that your daughter is going to die in two years, although I completely understand where your coming from. My husband and I did the same thing, read too much on the internet, and thought I was going to die. This site helped me a lot in coming to the realization that people live long and prosperous lives with this disease. We all may have to tweak our daily routines a bit, but this disease is manageable and survivable.
Best of luck to you and your daughter. I hope you get some answers from your doctors soon. Feel free to ask as many questions as you can think of, everyone here is very knowledgeable and know firsthand about Wegs.

[maria garcia]

I am very appreciate of all your answers. Ilive in Miami FL and my daughter is seeing a rhema.here locally. He is soppose to be very knowledgable in his field but no matters. Im very scarred right now. Has anyone had scleritis of the eye? My daughter is going back today but there corelating this with her PPANCA +. How would I know when this disease is in remmision> Do the panc test come out negative?Has anyone had False positive? Im sorry about hese questions my daughter does not want to answer anything. He said he is only there to treat the Dx. Very rude but bcse of my insurance I am stuck on my own finding infor. from the internet.:sad:

[maria garcia]

NIcole you have been an ANGEL, thank you for your kindness and knowledgable information. I feel so much better. I think Im just overloaded with internet information.I have read horrrible things llike lung hemmoraging etc. I had never heard of this immune disorder. I always heard of Lupus scleroderma Crohns but not P PANCA this is why im so confused. Is it true that this auto immune is the worse one to have? What has been your diagnosis so far. Its looking like my daughters is Scleritis of the eye. which is what took us to see the Rhemo. My email is INZINES at aol.com. If anyone in this forum wants to write to me. I wish this was happening to me and not my daughter.

[Red]

I am very appreciate of all your answers. Ilive in Miami FL and my daughter is seeing a rhema.here locally. He is soppose to be very knowledgable in his field but no matters. Im very scarred right now. Has anyone had scleritis of the eye? My daughter is going back today but there corelating this with her PPANCA +. How would I know when this disease is in remmision> Do the panc test come out negative?Has anyone had False positive? Im sorry about hese questions my daughter does not want to answer anything. He said he is only there to treat the Dx. Very rude but bcse of my insurance I am stuck on my own finding infor. from the internet.:sad:

i have scieritis that has resulted in thinnining of white part prednisolone has helped but that has probs to now on protopic ointment the dr i seen was top doc at University of Minnesota i hope this helps. P.S Get to a eye dr right away

[Sangye]

Hi Maria, welcome to the group. I'm sorry for what your daughter and you are going through. Her doctor sounds awful and definitely needs to be replaced. The info the others have posted is all excellent. (Well done everyone! :thumbsup: )

Since her involvement does not sound life-threatening at the moment, it would be a good idea to travel to a major center to have her properly evaluated and a treatment plan arranged. They will answer all your questions and give you excellent and trustworthy advice. She will not have to travel there often and can see local docs who will follow the center's instructions.

Would you be able to do that?

Meanwhile, here is the thread Phil directed you to: http://www.wegeners-granulomatosis.com/forum/general-wg-chat/986-what-newly-diagnosed-need-know.html

[Red]

I have scieritis of rt eye please see an eye dr. as soon as you can my eye got worse very fast being treated by top dr.

[maria garcia]

My problem is my insurance doesn't cover:( but I am of course willing to do whatever i have toIs there a specialist just for this disease. Is there a major center? Has anyone had nausia with alot of saliva spit up. she just told me this yesturday. She is spitting a mouthful of saliva(sorry if it sounds gross). No blood. Im thinking it might just be her nerves bcse of all happening so fast she was just diagnosed this week. Anyone heard of this happening?

[Sangye]

Does your insurance cover Mayo Clinic in Florida? They don't have Wegener's specialists but can work with the ones at Mayo in Minnesota.

The nausea and excess saliva sounds like nerves-- you know how you salivate more just before you're gonna vomit?

[NicShaf]

NIcole you have been an ANGEL, thank you for your kindness and knowledgable information. I feel so much better. I think Im just overloaded with internet information.I have read horrrible things llike lung hemmoraging etc. I had never heard of this immune disorder. I always heard of Lupus scleroderma Crohns but not P PANCA this is why im so confused. Is it true that this auto immune is the worse one to have? What has been your diagnosis so far. Its looking like my daughters is Scleritis of the eye. which is what took us to see the Rhemo. My email is INZINES at aol.com. If anyone in this forum wants to write to me. I wish this was happening to me and not my daughter.

There are bad things that could happen if Wegs is not treated, but it sounds like your daughter is in the very beginning stages of Wegs. This is good for treatment and recovery. Getting a good doctor on your side and starting some sort of treatment asap is the most important thing to focus on right now.
Many people have never heard of Auto-Immune diseases. The only reason I knew what they were is because my Dad was diagnosed with one about 10 years ago...but 10 years ago, we were blown away that such a thing exsited.
In my personal opinion, I don't think Wegs is the worst of the AI diseases. It's not always fun, but there are worse things out there. The fact that Wegs is treatable makes it better than some others, I think.
I was diagnosed in December. I was hosptialized with what they thought was pneumonia. I have had sinus and lung involvement, luckily no kidney involvement for me. I started Prednisone when I got out of the hospital right before Christmas, and as of the first of the year, I started on Cytoxin. Things started improving within weeks of starting treamtment...so things will get better for your daughter too.

[BrianR]

Maria - If your daughter has Wegs remember that it is treatable and she will almost certainly live a long and full life. You will both have to manage the disease, the doctors, and the medicine and you will certainly have ups and downs but it will be OK. I know exactly how scared and totally freaked out you are; I was there too when my wife got sick almost one year ago. Stay strong for your daughter, stay informed and diligent and it WILL be OK.

[Chris G]

Maria - I'm also a mom, and I can't imagine how scary it must be to have a child with WG, and be given no information from the treating doctor. Please try not to panic while reading all of the worst case scenarios that are found on the internet.

I am P-ANCA positive (MPO), as well. It seems that P-ANCA WG patients are rare. My symptoms are limited to my head an neck area. And, it may be of some comfort to you to know that I've read studies that say the those of us who are P-ANCA positive, have fewer organs involved (less likely to have kidney involvement).

Yes, I have had scleritis, and there were other eye symptoms too. Other ares affected are: sinuses, ears, throat (in the form of a cough, and occasional loss of voice), and some mild shortness of breath. Each of the two times I've had known flares (07/08) and currently, my P-ANCA tests were positive. During the time in between, when I had some strange self-induced remission, the P-ANCA was negative. So, for me, the ANCA tests seem to be an accurate indicator of disease activity - but this is not the case for everyone.

Please try to find a specialist that's somewhat near your home. You may have to travel there, but your peace of mind, and the care they will give your daughter, it will be well worth it.

Stay strong.

[drz]

I had scerlitis a few weeks before I found out I had Wegener's disease which was most likely the cause. The eye doctor was the first doctor to correctly guess I had some underlying systemic disorder like Wegener's which caused the scerlitis. He was successful in treating it and it cleared up in a few days. Further tests and evaluation will help clarify your situation and what treatment is appropriate. It sounds like you may need to find another doctor more sensitive to your concerns and then things should settle down some for you.

[maria garcia]

Chris Im so glad to find someone who also has PPANCA and MPO positive. Do you have Panca C? I was just at the eye doctor and they don't think is scerlitis bcse she has no pain just a bit of discomfort. I have to get a second opinion per the eye doctor bcse she want us to be 100% sure but she does not think it is. I see you also had eye problems not just scerlitis. I can't wait until her chest and sinus results come. How did the doctor diagnose you with wegners. What medications did they put you on? Im glad to have found this forum everyone has been so helpful and positive. I have learned so much from all.

[Chris G]

Maria - I'm not sure what you mean by "Panca C". I am not C-ANCA positive if that's what you mean. I am P-ANCA positive (MPO), like your daughter.

It could be that your daughter has episcleritis, which is inflammtaion of the thin layer which covers the sclera. It's not usually painful, but can be equally red and angry looking.

Here's a little section from one of the studies I mentioned:
The sensitivity of c-ANCA in active Wegener’s
granulomatosis is 91% and has a specificity of 99%3. The
patients with Wegener’s granulomatosis, who are p-
ANCA positive, have fewer organ involvements than
those with c-ANCA; the combination of respiratory tract
and renal involvement occurs less frequently in the p-
ANCA group. p-ANCA (MPO-) is found in fewer than 5%
of patients with Wegener’s granulomatosis.

As this study mentions, P-ANCA positive WG patients are less common, and may not have involvement of the lungs and kidneys. I do not have lung or kidney involvement. While it's certainly not a guarantee, your daughter may not either.

In 07/08 I had repeated sinus problems, an ear that would not unplug, with diminished hearing in that ear, croupy cough which lasted for 3 months, severe fatigue, daily headaches, and I kept losing my voice for no reason. The eye symptoms were episcleritis, scleritis, keratitis, eyelid swelling, and corneal infiltrates. I saw no less than 6 different doctors, and the only one that suspected autoimmune diseases, was the opthalmologist who was unsuccessfully trying to treat my eyes. He was the one who ordered an ANCA test on me. It was positive for P-ANCA, and the MPO was 98. I was still not diagnosed at this time, as very shortly after that, the disease went quiet on its own. It returned last year, starting with fatigue, and a plugged ear again. My symptoms this time were slightly different. But I knew I needed to get to a rheumatologist. This time I do not have eye involvement, but I have more nose involvement - crusting, bleeding, burning, etc, and the ear inflammation is also much worse this time.

I was put on methotrexate and prednisone, along with other standard suppliments. While the mtx has helped, unfortunately it's not going to put me into remission. I will be changing to rituxan in the next week or 2.

WG is treatable and manageable!! I hope that you are able to find a specialist quickly. It will speed the process of diagnosis/treatment, and also ease your mind immensely.

[maria garcia]

Did you ever have a chest exray and sinus xray ?

[Chris G]

Yes, however x-rays by nature, don't usually show much detail. In my case there wasn't much to see anyhow. You may need CT's to show better detail of both areas.

I've had both x-rays and CT's of the lungs and the sinuses. Nothing much to see in either place. Lung CT showed one tiny lung nodule, which may or may not be related to WG. Sinus CT showed mild mucosal thickening, but no damage.

Did you say that your daughter had some nose bleeding? A scope of the nose is a good diagnostic test.

[maria garcia]

Chris with no diagnostic test coming out positive for Wegener's and C- Anca was negative are they sure your diagnosis is Wegeners Granuloma? Wow, how confusing if the xrays are not a good detail. This disease is becoming so complex to understand. I want to make sure if they putter her on heavy meds there sure of the diagnosis. I mean yes she is p anca positive. But all in our family have suffered from allergies how scarry they come out with a false positive or vise versa.

[maria garcia]

Why is MTX not going to put you in to remisssion?

[Sangye]

Mtx can induce remission only if the disease activity is mild. Otherwise they need to use a stronger drug like cytoxan or rituximab.