What else can it be?

[makabe]

Unfortunately, I seem to be stuck in the diagnosis process. I know this is a hard disease to pin down and was wondering what else your physicians considered before deciding Wegener's was the culprit -- especially if you did not seem "obvious."

I have had a nearly daily fever for about 19 months. I have roving joint pain (hands and hips are the worst.) I have had almost nonstop SEVERE sinus problems that are almost impossible to treat with antibiotics (on Sunday, I finished six weeks of Bactrim and Levaquin that finally brought me relief, but I was symptomatic again by Monday afternoon and am now back on Levaquin.) My CT scans show 100% occlusion of my left side, which was clear when I had another sinus surgery just 10 months ago. I have really dry eyes and what has been called "recurrent corneal erosion."

I had a positive c-ANCA in September (but low titer.) My titer is now negative ( have been on pred but nothing else other than antibiotics) and I have NEVER had any other elevated inflammation markers.

I am scheduled for a sinus biopsy in three weeks.

So, if you made it this far... I'm trying to think of what else this could be if NOT Wegs. All docs so far think it is, but seem determined to find hard proof before treating. (I am trying to get into Hopkins. Also trying to see someone that has just moved to my area from the Boston Vasculitis Center.) What else was considered when you were diagnosed??

Thanks for reading!

[Chris G]

It sounds like wg to me. I can't think of anything else that explains your symptoms. If it walks like a duck and quacks like a duck.........

When I was diagnosed, relapysing polychondritis was considered. But you don't seem to have symptoms of that at all. I refused sinus biopsies because they are notoriously inconclusive. You can have negative biopsies, and still have wg. In the end, due to the overhwelming abundance of symptoms that pointed toward wg, that's the diagnosis I was given. And with all of the research I had done (like you have), I agreed completely.

[makabe]

Oh, my goodness, Chris, your first sentence is almost WORD FOR WORD what my main doctor has said. Everyone agrees is almost can't be anything else. But, because they don't see a lot of Wegs, they seem reluctant to pull the trigger on treatment. Meanwhile, I deal with all of the icky symptoms!

I feel better just hearing someone else that knows what I'm talking about say that. I'm sure you understand: I don't WANT Wegs, but I'm sooo tired of not having a clear course of action!

[Nancy-S]

I'm very interested in this thread. I walk and quack like a duck. But the specialist in Utah told me he doesn't see wegs and sent me home.

Since I've been home, I've had my first ever (in my life) sinus headache/infection. It was so intense I was up for 72hrs. The ear pain was excrutiatin. Finally I blew my nose, air literally squeeked out of my ears and I got relief. My hearing has significantly decreased since. Pressure still in my head and constant ringing in my ears. Now I have a flaming hot throat infection. Roaming leg pain, fatigue, and still traces of blood in my urine (last test was Nov).

I spoke with dr. Koenings resident dude yesterday. He changed his tune a little when I told him I'm very suspicious of the new sinus problems simply because I have no hx. I also reminded him the extent of his exam was to peek into my nose and ears and he looked at 6 week old labs.

I'm seeing an ENT this Friday. I think its wgs and I'm not giving up until I get on track to save my life. Sorry for so much detail but I want this story on here in case it helps someone in the future.

I've researched a ton, I've asked others to research (without emotional involvment) and came up with NOTHING. I did find a published paper from 1989 that studied a group of wgs patients with a medium age of 44. A good percentage didn't have kidney involvment and it delayed dx by 3.6 years. These patients typically had normal inflammatory markers. It was a good example that each patient is different and should be treated as such.

If you come up with other possibilities, I would appreciate the info.

[Chris G]

Karen - if you're not seeing a true wg specialist, you really must get one. Even if your doc decides to "pull the trigger" so to speak, he's probably not really qualified to treat wg, and will treat you "by the book", which is not the way to go - especially since you likely have "incomplete" or wg lite" like Sangye likes to call it. I have "limited" or "head and neck" wg (wg lite I guess), and it was not responsive to the initial treatments they started me on. You need someone who knows the disease, and can think outisde the box. Getting a diagnosis is very difficult, and I completely know your frustration and eagerness to simply find a diagnosis! For me, it was 4 years before I was diagnosed. Not until I saw truly qualified docs did I get the diagnosis.

Nancy - I too have always had normal or near normal inflammation markers. However, I've always had positive ANCA results while flaring. We are truly all different, and the lab results are not always a true indicator. But they are sometimes good for pointing docs in the right direction.

[Al]

You see, Nancy and Karen, in so many ways it doesn't matter what you call it; the fact is, when you hurt, you hurt. But a DX can indeed be useful, as the doctor usually won't start on a course of treatment without one.

Nancy, you are so right: "each patient is different and should be treated as such." And every disease is different. It is true that kidney involvement provides a greater sense of urgency (in my case, it was maybe three weeks between obvious kidney distress and the diagnosis). And it is also true that some signs are ambiguous, or some kinds of confirmation yo would like to see are non-existent. In your case, however, it is not clear that you do not have some kidney involvement, whatever they want to call your ailment. But I suspect that in many cases, the problem is as Karen notes: Doctors are reluctant to make the diagnosis, simply because they are not so familiar with WG. I know though sinus biopsies are not totally reliable, but often the pathologist is your best ally. (In my case, my nephro consulted with the pathologist before making the call.)

Al

[Nancy-S]

I am glad that we aren't diving into dx/treatment. But, wishing there was a way to intercept in such early stages. Surely remission would be better achieved. I suppose there's just not enough research. No $$, no time, no interest.

For that reason, I'm my best advocate. Ill keep a watchful eye, and count the beautiful days without harsh drugs. Your story (and so many others) about being 3 weeks from dialysis is the exact thing I'm trying to avoid. Currently my last labs were in November, much too long ago.

Karen, keep being assertive. It sounds to me like you're close! I hope its not what we think it is, and they can pinpoint something much easier to handle. It sounds like you're in good hands.

[makabe]

I was probably too hasty when I said "they don't see a lot of Wegs." I sought out doctors who do see Wegs patients regularly while I wait to hear about Hopkins taking my case. My doctor was supposed to call to follow up on that today. I just meant it's a rare doctor that has a Wegs-only specialty (or so it seems... I know a few exist.) While I wait, it turns out someone from the Boston Vasculitis Center has come to my area, so I'm also trying to get in to see him.

My husband made a good point tonight. He said there may be some element of them having to do certain tests before treatment, no matter sure they are, so they "cover themselves" if, heaven forbid, they gave you drugs like CTX or MTX when you didn't really have WG.

[Dryhill]

When I read stories like Karen's and Nancy's I realise how lucky I have been in being diagnosed so quickly. Ok so it took about two years for my GP to send me to hospital, the hospital did not like my SATs (78%) so decided to keep me in and start doing some tests, on the sixth day I first heard of this weird disease and treatment started.

Although my consultant is "only" a rheumatoligist he has fifteen of us with WG and patients with the other forms of Vasculitis. Whilst he is not a Vasculitis expert he does have a great interest in such diseases and attends relevent conferences and keeps abreast of the latest reports. Also Peter (PWC51) and I give him anything we have come across with a "Oh have you seen this?"

Karen all I can say is quack, quack and tell your doctor to pull his/her finger out. About a year ago, in this country (the UK), a 14 year old girl was diagnosed with WG. Two days later she was dead, purely because they had taken so long in working out what was wrong with her!

Jim

[makabe]

Every doctor alludes to the fact that they need to make a diagnosis because it's not safe to let Wegs go, but I appear "too healthy." The other perplexing thing is that I had a seemingly similar episode in 2007 that went away without treatment.

[Thakator]

Yes, Karen, your history and symptomology certainly makes us think of Wegs. Mine is primarily in the lungs so I can't comment with any first hand knowledge regarding the upper respitory system. I would, however, like to speak about your fever situation. As there are multiple potential causes of your other symptoms, so too are there multiple reasons for the protracted history of fevers (and, 19 months is an exceedingly long time for that).

Ten months of fevers which ultimately became a daily event was one of my primary symptoms - - the one that all of my doctors were the most concerned about. I was told by an Infectious Disease and Fevers specialist at the very outset that they would test me for every conceivable infection that applied to someone who hadn't been travelling in certain parts of the world. But, he also said that he doubted very much that it was an underlying infection that was the problem. Even though infection is by far the leading cause of fevers (which is why all the tests) and they had to positively rule that out first, he told me that an infection-induced fever rarely lasts longer than two or three months and that's why he suspected something else. Furthermore, he said the reason they don't usually last longer than that is because, "You either kill it by then (w/treatment I guess) or it kills you."

Several of us on here have had fevers as part of our history leading up to the dx of Wegs, but not all of us. Your fevers may, therefore, be a harbinger of Wegs or they may indicate another problem. Either way, that is a terribly important symptom that must be reconciled with its cause (for the causes are few and none are good). Given my personal experience and reading your story I say Wegs or some other form of vasculitis - - but I am most asssuredly no doctor, so my conclusion should be given absolutely no weight. But, I did want to relay my fever doctor's slant on things. He went on to say after expressing his doubt re: infections that the next most likely cause of protracted fevers would be certain types of cancers. The primary suspects there would be kidney or adrenal cancer simply because they often present w/fevers (especially the kidney one). Then he uttered a word that no other doctor had yet mentioned to me, autoimmune. He said that if my protracted fevers were not caused by an infection or by cancer then he believed it would prove to be an autoimmune sysytem disease that was the culprit. That's it, Karen, according to him someone with 19 months of fevers would not likely have an infection as the cause and if there is no other reaason to suspect cancer then it's autoimmune. No other categories. Which, presuming you have been or will be cleared on the cancer front, would lead him ( a fever expert) to conclude that you have an autoimmune disease and this, in turn, lends creedence to your growing conclusion that you have met up with Wegs.

He set me up with a rheumie as soon as all the cancer tests were complete. Bingo! She knew right away that I was a Weggie. I hope that you're not, but it sure sounds like you are. Please, either way, keep pressing for the cause, because - - as the rheumie said as soon as she told me she thought I had Wegs, "If I'm right, we've got to get on this right away (meaning treatment) because this can be fatal." She also said that the treatment itself could be fatal and that's why so many doctors are reluctant to start it without a positive biopsy. But her point was clear, if it's Wegs you do not want to delay treatment unless there is an overriding issue such as imminent surgery. It's the same conclusion you've already reached and I hope that my comments regarding the fever side of your situation will prove to be of help to you as you fight through this thing and as you try to understand all that's happening to you. Ron

[me2]

I don't have much to add to the subject here as so many good comments and points have been made. I went for months being treated for 'sinus infections' in pain and agony until and ENT took a sinus biopsy and bang- I had WG. These thirty plus years my inflammation markers have been almost useless, ANCA totally useless.

I would ask my doctor this "Given that there is no one test that is 100% reliable for WG, at what point , and how , do we decided it is or is not WG - are you waiting to do an autopsy to decide Dr?"

You can see now why I get in trouble with doctors from time to time. You might leave off the last part of my question and get better results.

Don't give up because they keep saying "I don't know". YOU know something is very wrong. Keep up the quest until YOU are satisfied that a reasonable diagnosis has been made and a good course of action to treat it.

Like my WG doc from thirty plus years ago, that I still see for other stuff, told me once "Nobody cares as much about your health as you do". He meant the comment to encourage me to keep educating myself and be pro-active in my care. Plus I found it mildly amusing.

[me2]

Wow, interesting story Ron , thanks.
Kirk

[Thakator]

Hi Kirk,
Yes, the old Wegs is very much a nasty disease - - but, we must admit it certainly is an interesting one! Ron

[me2]

Hi Ron,
Ha, yeah, I frequently tell friends "You know, if I didn't HAVE this disease , I would find it to be quite fascinating"

[Sangye]

Karen, knowing what I know about JHU I wouldn't worry too much about things right now. They've seen every variation on the Wegs theme there is. I know they're gonna be able to diagnose you properly, whether it's Wegs or something else.

As far as my own diagnosis, my inflammatory markers were fine until shortly before dx, though I was certainly not fine. I was crippled with severe joint pain body-wide, my lungs were massively but silently hemorrhaging and I was severely anemic. I could barely breathe or function. My delay in getting diagnosed was due to my own stubbornness in refusing to see a medical doctor.

[me2]

Wegener's is what they give you to treat you for 'stubbornness' Sangye. I know because it is what I was given for my own very intractable condition of stubbornness. I am in 'stubbornness' remission right now. I do suffer from flares from time to time.

[makabe]

This is all so helpful!! Thank you for taking the time for such thoughtful replies.

When I first developed a fever a few years ago, they looked all over for malignancy. They thought Hodgkin's at first. Then some other lymphoma. All of the cancer-related causes were ruled out and they don't seem to be looking for malignancy again since they figure more symptoms would be obvious by now.

I see an infectious diseases expert that, too, feels the fever is the thing to be most concerned out. He has had me officially listed as "fever of unknown origin" for ages. He, too, is the least reluctant to move forward based on symptoms since he has seen me the longest. The rheumatologist is the one really pressing for biopsy and they seem to be in agreement at the moment. I don't mind doing it, I just hope it's not one more thing to be added to my list of inconclusive tests.

That makes me think of something else. I had a sinus culture on 12/23 in the midst of the worst sinus "infection" I've ever had. The ENT said I was completely "full" yet the culture showed no growth. Could that mean the total opacification is granulomas and not infected tissue?

[mishb]

Hi Karen,

For me, I had 18 months of my GP telling me that my sinus infections were from allergies and hayfever (I am a hayfever and blocked ear sufferer since early childhood), my joint pains were from rhuematoid arthritis and that my Vitamin D levels are so chronically low that it could be the cause of all my pains, my sinus, my ears and eyes.

After trying every antibiotic & anti inflammatory under the sun, my husband finally talked my GP into sending me to an ENT. GP had never wanted to before because she said that she had it under control and she is giving the same strong antibiotics that an ENT would give. Thankfully an ENT moved into the same clinic that my GP is in and I got a cancellation the very next day. The ENT looked at my sinuses with a very long probe thing and said, I think you have Wegeners Granulomatosis. I couldn't understand a word he said because he said it with a german pronounciation. He sent me for some blood tests and he wrote a note for pathology about suspected WG and then I knew what he was talking about......thanks to google.

A week later, the results came back with positive Anca and everything else high. He straight away booked me in for a sinus biopsy. I first saw the ENT in August 2010 (18 months after first goint to my GP, but not since my first aches and pains, I just put them down to age) and the biopsy was performed in February 2011 (no insurance, so long waiting periods). The result was one very small granuloma but it was all that was needed.

Still with no insurance it took until April 2011 for me to get into the Rheumatology/Vasculitis Clinic at one of Melbournes largest hospitals......at this stage I was still only on strong antibiotics for my sinus infections and anti inflammatories for my RA. The ENT didn't want to give me anything until I saw the Rheumatologist.

I saw the head internist and he questioned the WG and even the RA diagnosis. He tested for Aids, Hep C and also TB. He sent me for xrays on my hands and feet and a chest xray.
I had these all done within the hour and then went back to him. He asked if anyone had done a urine test which the answer was no, so I did one of these also. Ten minutes later I'm being told that there is trace protein and blood in my urine and I would be admitted to hospital straight away. They started me on Pred whilst I was in the hospital, did more blood tests, lung scans and a lung function test.....the full grease and oil change you might say. I was in hospital for 4 days on just the pred and then the day I left they started me on MTX.

So I had just on two years before I started on any correct medications but now I seem to be back to square one with rheumatology, oncology, haematology and immunology thinking that maybe I don't have WG. A leukemia type was then ruled out and now they call it mixed connective tissue disease with RA and probably WG.

Either way (for me), it is still ANCA positive vasculitis and whatever it really is ......... it is still treated with the same medication, so I don't mind what name it is given.

Wow, I have never said all of that before.......it's not like me to go on this long ........I'm sorry :blushing:

What I'm trying to say, Karen, is that with all of your symptoms, whatever they decide in the long run that it is, you will more than likely be treated with the same medication and hopefully find a pain free existance after that.

[drz]

Many of us can empathize with your dilemma of feeling poorly with many symptoms and no one able to figure out why or offer much hope of relief soon. All you can do is push doctors for answers, ask questions to help them focus treatment plan and clarify their thinking on what info do they need to diagnose your problem. Suggesting they refer you to someone with experience in treating or diagnosing GPA might also help since many of your symptoms suggest this is possible diagnosis.

You are way ahead of many of us who never heard of GPA til we were diagnosed and only after having such a severe case there was little room for doubt on diagnosis, just question of whether we could survive the illness and/or treatment. Keep pushing for clarification of why you have these symptoms and keep asking what is their plan to get answers as to why?

Best wishes for better health soon.

[Al]

Karen--a "full" sinus without signs of infection are almost the definition of WG (one definition, anyway). And, per Ron's comments, I have no doubt that whatever you have is autoimmune. There are, to be sure, many variations on this theme. I never was feverish; even at the height of my very obvious inflammation, my temps ran cool. But major unexplained fevers do mean something dastardly is happening.

As I hinted at earlier, I recommend getting the biopsy, even if it is inconclusive, for the simple reason that the pathologist will have seen more of your kind of tissue than the other doctors, and can bring a lot of interpretive chops to the show.

Al

[Al]

Wow, Michelle--no, I don't think you have told us the whole story before. Thanks for sharing it, and no need apologizing!

Al

[Nancy-S]

Michelle, your story is valuable. Important, interesting, and valuable. Thank you for sharing!

[Sangye]

Wegener's is what they give you to treat you for 'stubbornness' Sangye. I know because it is what I was given for my own very intractable condition of stubbornness. I am in 'stubbornness' remission right now. I do suffer from flares from time to time.
ROTFL Exactly, Kirk.

[Chris G]

Every doctor alludes to the fact that they need to make a diagnosis because it's not safe to let Wegs go, but I appear "too healthy." The other perplexing thing is that I had a seemingly similar episode in 2007 that went away without treatment.

This is EXACTLY what happened to me Karen. First real flare was at the end of 07, into Feb of 08, and then it disappeared mysteriously without treatment, before I could get a diagnosis. That's what I've made mention of in other posts........that early on it's so tricky because it seems to come and go on its own. Until the day it comes and stays. That's when I believe for some, it's finally fueled up to stay and cause real trouble.

My early stage symptoms were very similar to yours. Eustachian tube dysfunction, causing balance problems, dizziness, fluid build up, ear infections. Many eye probs, including dryness, redness, eyelid swelling, an ulcer on my cornea, etc. Sinus headaches and pressure every day. I took so much motrin, I'm surprised my kidneys didn't fail. Like you, early on, antibiotics gave me some relief, but literally the minute I stopped taking them, the symptoms returned.

During that early stage, you're bounced around from specialist to specialist who try to treat each symptom as a separate issue, rather than putting the puzzle together. I was told I looked healthy..... I was told that if it was wg, it would not have gone away on its own......I was told that he could not see any active signs of wg. SEE?!?!? Really? Most of what we suffer can not be SEEN with the naked eye, especially to a doc who does not have wg experience, but doesn't have the guts to say that.

Keep pusing on Karen. You'll get there.

[maria garcia]

Positive Anca defenitely and your symptoms defenitely I would say Vasculitis it doesn't have to be Wegners there is also MPA wich is what they think is what my daughter has and there are more under positive Anca's. There all treated the same and do about the same to the body. My daughters rheumy kept telling us its just a marker and that my daughter did not have vasculitis until she finally had a biopsy and vasculitis came back. Thats what made her Rheumy finally admit yes she has an illness and its vasculitis most probably MPA and put her on Meds (MTX)(pred).

[Sangye]

Never let a doctor tell you it can't be Wegs because you "look too healthy." When my lungs were hemorrhaging with a flare in 2009, several JHU docs who weren't Wegs specialists concluded it wasn't a Wegs flare because I "looked too healthy." They discharged me from the hospital with a dx of bronchitis. When Dr Seo found out first thing the next morning he called me to come in immediately.

[Chris G]

Never let a doctor tell you it can't be Wegs because you "look too healthy." When my lungs were hemorrhaging with a flare in 2009, several JHU docs who weren't Wegs specialists concluded it wasn't a Wegs flare because I "looked too healthy." They discharged me from the hospital with a dx of bronchitis. When Dr Seo found out first thing the next morning he called me to come in immediately.

A perfect example Sangye!!! Yet they do it to us all the time!! It's infuriating!!

[Sangye]

They just don't know. If all they know is the textbook description of Wegs then it's logical to assume the person would look as unhealthy as they are. I was in the JHU pulmonary unit at the time. Many of the JHU docs had probably only seen Weggies looking the way I did when I was dx'ed. No one would have said I looked healthy then!

This explains why, but it doesn't make it okay.

[delorisdoe]

The pumonologist that diagnosed me said exactly that. He told me all about wegeners and I just sat there mesmerised by how handsome he was. I had already read the internet discription of wegeners that he was reciting to me so I did not really have to listen. He mistook my silence and i guess my stunned look on my face for dispare because he left the room and then came flying back in and told me "you know, i dont usually see people with wegeners until they are in the icu fighting to stay alive". He was trying to shock a recation out of me. I think I just thought of how pretty he was.

[Sangye]

ROTFL Leigh! http://www.smileyhut.com/laughing/rofl.gif (http://www.smileyhut.com)

[delorisdoe]

He really is good looking.

[Chris G]

The pumonologist that diagnosed me said exactly that. He told me all about wegeners and I just sat there mesmerised by how handsome he was. I had already read the internet discription of wegeners that he was reciting to me so I did not really have to listen. He mistook my silence and i guess my stunned look on my face for dispare because he left the room and then came flying back in and told me "you know, i dont usually see people with wegeners until they are in the icu fighting to stay alive". He was trying to shock a recation out of me. I think I just thought of how pretty he was.

Haha!!! Love it! But, I have not had that ......um......problem.

[Chris G]

He really is good looking.

So you see, we never have to worry about Leigh skipping a doctor appointment!!!

[pberggren1]

My last Wegs doc said: You can't be flaring, you look too good. I walked out the door and said: It was nice knowing you.

[Al]

Haha!!! Love it! But, I have not had that ......um......problem.
This made me laugh, Chris! (After, of course, laughing at Leigh's deal....) I haven't had that problem either. But I was somewhat amused that, at my last appointment, when Drac walked in, I caught a strange, fleshy sight in the corner of my eye. Turns out he was wearing shorts--and a huge ankle boot-brace. Turns out he was cleaning out his flooded basement, slipped, and shattered the poor ankle. It wasn't really funny, but we did have a bit of a chuckle. I have always claimed the right to be a Sick Old Man; for a while, he can use the same excuse. Hey, I wouldn't have responded to a golden-haired Adonis anyway.

Al

[delorisdoe]

So you see, we never have to worry about Leigh skipping a doctor appointment!!!

You spoke way too soon on that one. My wegs doc and my pulminologist are no longer in the same wing of the hospital. Now unless i get pnuemonia again or a bad lung flare up I may never see him again. My wegs doc is anything but pretty. He is quite hairy

[Sangye]

So the only way we're gonna get you to see your doctor is if we send you a case of razors?

[freakyschizogirl]

I'm gutted - i dont have any handsome Doc's - maybe i've got the wrong doctors or wrong disease?

Some nice looking (male) nurses who take my blood tho...Had a nice chat with one about tattos and how veiny his arms were - very impressive (if you're into that sort of thing....)

[delorisdoe]

I do find Tommy Lee to be quite attractive...