Help regarding diagnosis??

[cnorth]

Hello, to all.

I am new to the forum and I am in Western Australia. After repeated bouts of eye inflammation, ear pain, sensory neuropathy, visual issues, tingling and numbness in my arms, hands, legs and feet, sensitivity to cold, inflammation around the exterior of my nose, sore throat/losing my voice, strange wrinkled skin (and the list goes on) my doctor decided to run a test for ANCA. The test came back positive for
cANCA, but negative for PR3 and MPO. He says that I don't have Wegs and that I will never develop it? Is that possible?? I have read that if you test positive for cANCA and negative for PR3 and MPO, you still have the disease but may be in remission?? My inflammation markers were elevated in May, but since I had what I call an "attack" (the last week in July, when the ANCA blood test was taken), where I had partial paralysis of my arms and legs and was put in hospital...strangely my inflammation markers and all other blood test results have been within the normal parameters.

Has anyone had an experience like this, or does anyone know much about cANCA and PR3 and MPO? My doctor is very stubborn and won't refer me on to anyone as he believes that I don't have the disease, however he hasn't really taken into account my symptoms, he is just looking at the blood test results. Meantime I am continuing to suffer with these problems and feel that I am deteriorating.

Any advice would be greatly received. As would anyone out there that may know of someone in Perth, Western Australia that is experienced in dealing with Wegs. I guess I need to definitively rule it in or out and so far the doctor I have seems to know very little about it.

Thanks for allowing me to share on your forum.

Cindy

[JeanMarie]

Hi Cindi,

Positive ankas are not necessary for a diagnosis of Wegener's or
vasculitis. However you do have a positive c anka so I don't
understand what your doctor means. Is he a rheumatologist? At a
minimum, you need a consult with a knowledgeable rheumatologist.
These are very rare diseases and many doctors have never seen a case.
Here are a couple of sites that give info on diagnosis that might
be helpful to you:


Granulomatosis With Polyangiitis (GPA) (http://www.niaid.nih.gov/topics/gpa/pages/default.aspx)

Vasculitis Foundation » Frequently Asked Questions (http://www.vasculitisfoundation.org/education/faqs/)

https://labtestsonline.org/understanding/analytes/anca/tab/test/
As you can see from the 1st site, diagnosis is
complicated & you need more than a blood test. (We are all
different—but just as an aside I was originally diagnosed with
limited wegs without a biopsy)

We have some Australian members, and I think someone will have
info about doctors for you.

In the meantime, I hope you find answers and successful treatment
soon .

[JeanMarie]

Hmmm--sorry, I lost a few lines from my post. I wanted to say welcome & I'm glad you found us & I think the folks here will be able to help you.

[Pete]

Hi Cindy,

Can you get another doctor on your own, or are you stuck with this one? Some of the Aussies will likely chime in soon with suggestions.

The ANCA tests are not sufficient for diagnosis. The most accurate diagnostic is a biopsy of the affected tissues to see if granulomas are present. This is an invasive procedure that usually requires general anesthesia. Symptoms can also provide clues.

The best advice I can give you is to find a doctor who has experience treating Vasculitis (wegs is one of over a dozen forms of it) and follow his/her treatment plan. I doubt that your doctor has much experience treating Vasculitis (and you should ask).

Good luck, and let us know how you're doing!!

[drz]

If he is adamant that you don't have Wegs, what is his diagnosis that would explain all your symptoms? There are many types of autoimmune disorder and you may well have one (it might not be Wegs) but it seems you will need a better doctor to get a correct diagnosis and appropriate treatment.

No good doctor will tell you that you will never develop ​a certain disease since no one can predict that. I would also strongly encourage you to get another evaluation from some one with more experience and skill in treating autoimmune disorders on the likely chance that is what you have.

If the lab results are correct they would be a little unusual for an active case of Wegs. Often ANCA tests are negative in people with Wegs but a positive ANCA test should have some PR3 85% of the time, but that means in 15% it doesn't. The numbers for MPO run lower. But the absence of either PR3 or MPO is a positive thing in that those people had a higher survival rate in one study. See these links:

https://labtestsonline.org/understanding/analytes/anca/tab/test/

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2684710/

My doctor at Mayo wrote this article which also points out some things doctors should know about ANCA testing but many don't:

http://www.the-rheumatologist.org/article/acr-winter-rheumatology-symposium-tips-for-anca-testing/

[Birdie]

He says that I don't have Wegs and that I will never develop it? Is that possible??

Welcome Cindy,

Let's see if I understand from what you posted. You need a different doctor and you already knew that. You may have Wegeners or you may not but you knew that as well. You were told it's not Wegeners but you know to ignore that guy. Seems like you have a good understanding of your situation. Your understanding improves your odds of quick recovery. :) :) :)

I had most of your symptoms including "(and the list goes on)". When I realized I was rapidly deteriorating there was only about a month before suffering organ damage. Time is critical if it's WG so don't relax, don't accept "might be" something, most of all don't be ignored or put on hold.

Ask your questions here and there's bound to be somebody who knows the answer first hand. The answers come quick too.

Gary

[woz]

Hi Cnorth, you definitely have something going on, and the sooner you find the answers, and get the healing process going, the better, If your doctor is not being proactive, I would be getting a second opinion, I would be seeing a rheumatologist or someone in immunology, all the best.

Regards Woz

[cnorth]

Hello, and thank you all for your advice, comments and warm welcome. You have certainly given me the courage to push forward and find some answers as to what is going on. Just got a couple of things that some of you may know something about:
1. Did any of you have high liver enzymes with Wegs?? I have had consistent high ALP and GGT enzymes for about 8 years now.
2. My inflammatory markers were at 20 and 27 (ESR) in May and June, however strangely when I had what I call the "attack" at the end of July they were at 16 and 7 mm/hr. Have any of you experienced prolonged periods of high inflammation, and then have the symptoms appear much later? So in my case my inflammation was obviously up in May and June, however the effects of it didn't actually catch up with me until later in July (and consequently well after my inflammation had gone down). Here we are in September and I am still feeling the effects and have all the symptoms - Is that possible?
3. With Wegs after you have an attack and the inflammation goes down, do your symptoms settle and you feel ok again, or is it always a day by day battle, feeling terrible even after the attack is over?

Also I questioned my doctor as to why he didn't think I had Wegs given that I have a positive cANCA (even though the PR3 and MPO were negative). His reasoning is that apparently you can have positive cANCA without the disease, he said that apparently there has been a study done (here in Australia I think) in which 7% of the test population, tested positive to ANCA but had no signs of the disease, even when watched over a long period of time. I have no knowledge of this study (tried to look it up/google it, couldn't find anything) so I don't know whether that is true of not!

Sorry if these sound like really silly questions to you all, I am just trying to get a bit of a handle on what's going on. Thanking you all again.

Cindy

[Pete]

Hi Cindy,

Once a flare is over, you should start to feel better each day. The improvement may be slow. If there wasn't too much damage done, you might get back to pre-wegs health. Hope so for your sake.

[annekat]

Welcome, Cindy. I wish all your questions were easily answered. I have WG but at dx had only a barely positive anca and haven't had it tested since. A nasal biopsy proved I have it and all my symptoms are classic. It's true that flares and symptoms can come and go whether you are being treated or not. Many of us have had 'smoldering' WG for months or years before dx, where symptoms came and went and seemed like other conditions. If your doc is not a rheumatologist, or even if he is, I think it would be wise to seek a second opinion from one who has experience with WG and vasculitis in general. There are several Aussie members on here and some may live near you. I'd get in touch with Michelle, for one, who can connect you with an AU facebook group. Also there is a map link in blue at top of this page which will show you where others live, if they have added themselves to the map. Anyone on here would be open to a private message from you, I think. Maybe you can get the name of a doc or center where you could be evaluated and treated if necessary. And if your doc doesn't think you have WG, he should be doing more to find out what you do have! He has no business saying you will never develop WG. Stubborn doctors are the worst kind, IMO, and likely have caused considerable damage and deaths. Keep us posted and continue to ask any questions.

[Birdie]

3. With Wegs after you have an attack and the inflammation goes down, do your symptoms settle and you feel ok again, or is it always a day by day battle, feeling terrible even after the attack is over?

a study done (here in Australia I think) in which 7% of the test population, tested positive to ANCA but had no signs of the disease

Sorry if these sound like really silly questions

There is no such thing as really silly questions (however super silly questions are everywhere :) ). Don't be afraid to ask anything you need to know.

When it comes to Wegeners there are few absolute conditions or circumstances. One person has a flare and gets over it quickly while another person takes months or years to see improvement. Anything called normal is on a case by case basis. Normal for one person is playing basketball or mountain climbing. For me it's being able to get my wheelchair into the truck and drive to the grocery store. A few times a week I'm in good enough shape to do housework for a couple hours. I have hope of improvement. For now, a trip to the VA hospital like I did today... wipes me out for a couple days. But they did a bone density scan for some reason unknown to me.

I've seen statistics about the general population testing positive for c-ANCA which range from "a small percentage", all the way up to 20%. Also, Wegeners is not the only cause in a person who doesn't normally have a c-ANCA reading. Sorry I can't quote the other causes but as I recall it was only a small number of possibilities, 2 or 3 if my memory is correct.

Since you've got c-ANCA your best bet in diagnosis of WG would be a tissue biopsy. The exact granuloma in a tissue biopsy is only caused by two conditions and c-ANCA is not caused by the other one (pyostomatitis vegetans). Anything less than those two symptoms is not an absolute diagnosis of Wegeners. Makes WG sorta hard to diagnose for many people who have it.

Hope that helps, it looked like what you most needed to hear.

Gary

[JeanMarie]

Hi Cindy,

I've had high liver enzymes a few times—for me, it signals a
flare is coming. One of my befuddled doctors even ordered a
sonogram, which showed nothing. Another doctor thought it was a
reaction to an insect bite, since I had a rash at the same time.
Another jerk insisted I was an alcoholic. When I started this
journey, it took awhile to find competent doctors. I was told my
liver was pristine after the tests. The numbers go back to normal
after treatment.

Also, I think Giant Cell Arteritis (another form of vasculitis)
has this as one of its symptoms.

If 7% of the population had no signs of wegs than 93%
did—somethings wrong with the doctors reasoning!!!!!!!!!!!!!!!!!

Ask anything—that's what this site is for.

[txrepub4God]

I am a Wegs patient that has always had some very strange ANCA tests. It's funny - I am the one that finally found my own freakin' diagnosis, which is sad, but that shows you how hard to diagnose Wegs can be. I was continually sick, I mean, ALL THE TIME. From sinusitis to bronchitis to any other "itis" - I was always sick. I kept noticing my eosinophils were VERY elevated, which show you have constant inflammation in your system somewhere. My eosinophils were usually testing at over 10%, which from the research I did, points to another vasculitis called Churg-Strauss Syndrome. I had also had a lung infection called aspergillosis fungisitis, which on some website linked up with Churg-Strauss. Since I knew I was going downhill super-fast and my kidney function had dropped from 45% to 27%, I asked my kidney doctor to test for the Churg-Strauss. When my ANCAs came back, I was negative for both cANCA and pANCA, but my PR-3 and MPO were both abnormally sky-high. My nephrologist consulted with a pathologist friend of his, and she said it was Wegs.

However, after treating me for Wegs for all of 1 month, my nephrologist second-guessed both himself and the pathologist, and instead of sending me to a rheumy, he just took me off all Wegs treatment and that was it. I BEGGED him not to. I was bawling on the phone, pleading with him. Then I lost my health insurance from losing my job due to health issues, and I couldn't get any treatment at all for the next year until I got married. So by the time I had insurance again, got super sick again, begged doctor after doctor to test me for Wegs, I found my saviors at UT Southwestern Hospital in Dallas, TX. By this time, my ANCAs were showing positive cANCA and still abnormally high on the PR-3. Finally, they did a sural nerve biopsy and diagnosed the Wegs. I could KICK my kidney doctor, but he was afraid the Wegs treatment would damage what was left of my kidney and really negatively affect my health if it came to be that I didn't have Wegs. However, he could have KILLED ME by deciding himself I didn't have Wegs without getting biopsy proof before taking me off the medications for it.

My latest ANCAs show that I'm negative right now for the cANCA, but still abnormally high for the PR-3, although it has come down a little being on the Wegs treatment for 3 months. All I can say is, you know in your heart you have the WRONG doctor right now, and the wrong doctor can kill you. You are the #1 only person on this planet that has the highest reason to fight for yourself and your health, because without YOU fighting for yourself, who else cares as much as you? I have no problem leaving a doctor who I feel belittles me or my opinion, doesn't listen to me, or could possibly kill me with indifference. I matter, and YOU MATTER, so find a doctor who realizes you matter. :)

Welcome aboard, unfortunately!

[mishb]

Hi Cindy and welcome to this WG forum.

I have seen that some of the Western Australia members in our Aussie & NZ facebook group have given you some names.
I might bump your post back up to the top so that others who may have missed it, can see.

I hope you can get your doctor to organize a referral for you to one of the WA hospitals so that they can check things out.

[Margaret_B]

Dear Cindy,
When I was having many of the same symptoms you described, the doctors I sought to help me also reported that my blood work and chest X-rays did not show anything serious. Thankfully, the eye doctor treating my eyes for pain and inflammation KNEW that my symptoms were due to some auto-immune illness and, when I started coughing up blood, she insisted I find another doctor to help me get a diagnosis. She knew of a great Rheumatologist who successfully treated other patients of hers who had eye inflammation but did not know which disease was causing it.

Thankfully, the doctor I was sent to knew about Wegener's (I will call it hereinafter "GPA") and sent me to the hospital IMMEDIATELY, that very day (read my post under the heading "Medications- Rituxama", that I posted just a little while ago). There is a very small window during which the elaborate treatment I received can work the most effectively to STOP the inflammation and damage to the lungs and kidneys. PLEASE see another doctor who will consider sending you for a kidney biopsy, the only way it was proven I had GPA. I was awake during the procedure, under local anesthesia, on my stomach, with two interventional radiologists guiding two nephrologists ("kidney bean" doctors) through the use of a tiny IV camera, in order to remove two plugs of tissue from my kidney.

It is important to have blood work done to absolutely rule out that you have TB before treatment for GPA can begin. The blood test has to be done when you are not on steroids.

Your symptoms sure sound like GPA to me! I am not a doctor, but three doctors who I saw regularly and two emergency room doctors I saw when I had alarming symptoms could not diagnose me over a three month period using the results of their exams and blood tests, x-Rays and MRIs! GPA does not reveal itself in conventional ways and it is VERY dangerous to leave it untreated!

If you cannot get any results in Australia, and can afford to travel to the USA, try to get your doctor to refer you to Johns Hopkins University Hospital, Bay View Campus, in Baltimore, Maryland! Visit the Johns Hopkins Hospital website searching for "Vasculitis Center". You will also find information about GPA on that website.