Another newbie - Chicago area - Best friend DX'd with Wegeners yesterday (8 July 2010

[Cubby]

Hi Everyone,

I just found this forum last night after coming home from a specialist appt with my very best friend. I'm really trying to read up, learn, and find out how I can be a balanced center of support for my friend, her husband and adult children. We were both scared and I was so relieved to read here that people can live long healthy lives with this disease.

My friend is not an online person, and had mentioned she might look for a local support group. When I mentioned I have found online support helpful because it expands our limited access we have to info in our local area's she was interested and agreed with that thought process.

First I would like to ask if anyone from this forum is in the Chicago area.

I'd also like to explain a little about what is going on with my friend.
She has a dx, but an 'in remission' dx, because the blood test indicates she has Wegeners.
All of her tests were fine, she does not have the usual symptoms so she is 'out of the box'.
She did have a horrific ear infection and has scleritis but that is the extent of her symptoms.

A little about me. I am an avid advocate for missing and unidentified persons, and I am a member and moderator at a large online crime sleuting community. I don't know if it is ok to add the name of the site and I don't want to break any rules so I wont. I'm also a volunteer research specialist for a missing persons NFP. I love sleuthing, and helping families..... and cold cases. There is some joy in getting older 40+ year unsolved cases officially re-opened. So the mystery and trying to find pieces of the puzzle is in my blood. I'm also a Chicago Cub fan, so patience is in my blood too, lol.

I am looking forward to learning. If I don't post a lot at first, it is because I am trying to read and see if my questions have been answered somewhere in the forum.

I'm good at internet sleuthing and research, and am happy to help with searching for info if anyone gets stumped.

Thank you for letting me share and any info you can offer or suggestions are greatly appreciated.

Cubby

[andrew]

Hey Cubby welcome to the group, glad to have you aboard!

What was it in her blood test that indicated WG? I assume it was ANCA? You'll be nagged to do so over and over (especially by Sangye when she gets back online) but if your friend is not already seeing a WG specialist then that should be the next step. Someone else here may be able to recommend one in your area. Speaking of your area, check out Chicago Area Vasculitis Education and Support (http://chicagovasculitis.com/Homepage.html), it's a local Chicago Vasculitis support group that will probably be able to help.

[Cubby]

I will find out the name of the blood test. She did not tell me specifically which one, but said it is not a test in the normal panel of blood tests. This test would have to be requested specifically for this and is seperate. Whatever her number is, was supposed to be in the 100's? and she is at 360 or 370. which is considered a sky rocket of a level.


All of her other tests are normal. I am also hoping to get an idea on who the best specialists are in the Chicago area and making sure who she is seeing is the right doctor.

So far I have not been able to find if anyone here has been treated at UIC in Chicago.



thank you for the welcome and your help.

Cubby

[Jack]

Hi Cubby,
Sorry that you had to come to find us. However, from your description, the diagnosis of Wegener's would be considered to be a little premature by most. The problem is that it cannot be positively diagnosed from any blood test, only from cumultive probabilities indicated by a number of symptoms and test results. That is not to say that your friend does not have Wegener's, just that it would usually take a whole lot more symptoms or a positive biopsy result to confirm.

[JanW]

Cubby, the c-ANCA test is considered about 80 percent conclusive at best, so my assumption is that the rheumy is hooking that together with the eye and ear problems as evidence of WG. For instance, if you wiki scleritis the very first thing that comes up is its association with WG. The second is an association with RA. Ear problems are rife in WG patients, which is why we get yearly hearing tests and most of us are spending some amount of time with our ENTs.

Based on these clinical symptoms, plus information from the ENT, plus a blood test which I am assuming included c-ANCA and P3, I believe that a rheumy could be accurate in reaching this diagnosis. Sounds like he is treating her with methotrexate, which is commonly abbreviated here at mtx. This is a widely used immunosuppressant. They have had great success with it in RA patients, and are constantly looking for ways to use it to treat other autoimmune diseases. You will see it mentioned frequently here as a maintenance drug, after a patient has been on a course of cytoxin (ctx) or rituximab (rtx) at the point their disease was diagnosed (dx).

However, some patients do start on mtx. I am one of them. It also looks like the rheumy didn't put her on prednisone (steroids) which you will see that most on these forums are on in addition to their immunosupressent. I am not on that either, the reason being that my rheumy saw no immediate risk to my health if he didn't quiet the inflammation immediately, rather than taking several months to get my blood work back to normal. This is a fairly unusual stance for rheumys to take but seems to be becoming more common from what I am reading elsewhere, as both docs and patients are really becoming more concerned about the long-term use of steroids My disease progression, which you can read about in my introduction, started with subglottal stenosis and a saddle nose, and other than very, very minor sinus involvement vs. many WGers, I've had no other symptoms. My rheumy chose to start me on 15 mg of mtx, which I've been on since April. My inflammatory load (as measured by c-reactive protein - C-RP), sedimenatation rate (sed rate or ESR) and rheumatoid factor (RF) are now all normal again, although c-ANCA and P3 are still present.

Finally, the numbers on c-ANCA and P3 can't be relied upon to predict seriousness of the disease...I have a higher P3 than many on these boards, but am largely asymptomatic. A phrase you and your friend may hear from your rheumy in regards to this is "I don't treat the bloodwork, I treat the patient."

Hope this helps.

[Cubby]

Thanks Jack I agree with you. Her doctor did specify some would dx while others would not. In this case it would depend on which doctor. His term was in remission.

Jan, yes MTX is what she was started on. She had been on prednisone for several months but the side effects were such that it is not currently an option.

This is so new to me and my friend I probably won't know more until she has more info from her doctors. We've got lots more to learn and ask to get a better idea of what is going on with her.

Thank you both.

[Cubby]

Hi Cubby,
Sorry that you had to come to find us. However, from your description, the diagnosis of Wegener's would be considered to be a little premature by most. The problem is that it cannot be positively diagnosed from any blood test, only from cumultive probabilities indicated by a number of symptoms and test results. That is not to say that your friend does not have Wegener's, just that it would usually take a whole lot more symptoms or a positive biopsy result to confirm.


Thank you Jack.
Can you tell me more about the biopsy you mention? The doctor did mention she was out of the box since she did not have the cumulative symptoms. As I mentioned, the concern was the blood test. I'm curious about the biopsy and what it is about.

this is all so new to me, I'm not sure, and the doctor did mention it was kind of like a 50/50 based on how some doctors would or would not classify or dx this.

Maybe some info on the biopsy would help me understand better.
TIA.

Thank you.

[Jack]

I think that the biopsy has to be taken from an area that is actively involved and inflamed due to the Wegener's. This is usually sinus, kidney or lung. They are looking for the presence of Granulomas. These are spherical groups of cells that are particular to this disease and give a positive diagnosis. Of coures, if they fail to find them, this does not rule it out. It might just be that they looked in the wrong place. The system used for diagnosis of Wegener's is the BVAS test. This scores various symptoms and gives a probabliity of positive diagnosis.

I think that some symptoms are quite rare in conditions other than Wegener's so they immediately sound alarm bells. Perhaps the ones your friend is displaying come into this catagory.

[Cubby]

Thank you Jack, this really helps. I will make sure to mention both the biopsy of inflamed tissue and the BVAS test.

[Cubby]

A side note. I hope I do not sound too short with my replies. This is not my medical condition and I am hesitant to share too much about someone elses medical history and info. With the info everyone has shared I have some great questions to ask, and am on the fence with her DX.

By on the fence I mean, is this an accurate DX based on a high level blood test, and pretty much no other symptoms. At least where inflamation is concerned.

Both of us left the appt wondering how many mostly symptomless people have this elevated blood test level. It could be the luck of the draw to have found an elevated test early on and an indicator of what might come in the future?

Right now I am on the fence, and will present what I have learnt here.... for her to determine if she wishes to take this doctors word for it or seek another opinion.

TY all so much!

[Jack]

The blood test was probably the ANCA test. Try doing a search on this forum for ANCA in "Titles Only" and you will find several threads on the subject. It is a cause for debate on the forum because it used to be considered very important in the diagnosis of Wegener's, but now has lots of questions attached.

[JanW]

Cubby: Having a positive biopsy is considered the 'gold standard' and 100 percent, conclusively proves that you have WG if the granulomas are found. The problem is that biopsies can be done (as one was in my case) and you don't find granulomas, or you don't even get enough biopsy-able material. So a good doc would never 'not' make the call of WG if they couldn't get a biopsy that was positive for the disease.

That having been said, your friend has scleritis which in and of itself is rare and positively associated with WG. Also, ear infections. There are certainly many people who have posted on here who only have on or two symptoms, or varying severity who are being treated for WG (my serious symptom is subglottal stenosis but I have little of the systemic stuff that people here report.

I'm not saying that she has WG for sure, but the bloods (and the fact that c-ANCA isn't associated with other disease) with the rare inflammation of the eye and ears are adding up for the rheumy. In any case she is being treated with a very middle of the road immunosuppressant which hopefully won't cause side effects.

[Jack]

I agree with most of that although I would not dismis the possible side effects of Methotrexate.

[JanW]

Quite right, there definitely can be side effects, just hoping that she won't have any.

[Sangye]

Just chiming in to say Ditto. Ear "infections" + scleritis + positive ANCA points to Wegs. She needs a proper evaluation and dx by a Wegs specialist. If she cannot find one in Chicago, she should seriously consider traveling to Mayo Rochester or Cleveland Clinic (whichever is closest) for a proper workup. If it were me, I wouldn't trust any non-Wegs specialist who says she has Wegs or doesn't have it, or that it's in remission.

Proof via biopsy is not always an option. Sometimes they have to treat empirically and see how the symptoms respond.

[Col 23]

Welcome Cubby, hope your friend is going okay and has a confirmed diagnoses as soon as possible.
cheers col 23