Confused with pANCA test and doc recommendations

[lovetoswim]

Hi everyone, I'm so glad I found this website, although the more I read, the more scared I am. I, like so many on this forum, have led a clean life, no meat, no sweets, 2-3 hours of exercise a day for the last 30 years. My only issue was vertigo and sinus infections that lasted for weeks. Since January of 2016, I've had chronic sinusitis, and in December, had pneumonia, which I quickly recovered from, but shortly after finishing the antibiotics, I got a raging sinus infection and the flu. The pattern has been that a week after I finish the antibiotics and 5 pack prednisone, I start getting all the sinus infection symptoms back. Right now, my energy level is high, although I still have minor mucus discharge and tinnitus. I only usually missed 3 days a year of exercise, but in the last 3 months, I've missed an entire total of 1 month. Something isn't right. My family doc suggested I see an immunologist, and my pANCA was 1:3 and Lymphs were above normal. I went to an ENT to inquire about a nasal biopsy and he does sinus surgery along with it. I am opposed to doing that b/c he will put me on high doses of prednisone first and do the surgery, followed by more meds. If the nasal biop is negative, I will still need a lung biopsy. Is it possible to find a doc who will just do the nasal biopsy without the sinus surgery "package?"

Also, since i feel well right now, if the lung biopsy comes back positive, do I have to immediately go on those scary meds, even though I feel almost "normal?" I know my life is going to change, and not for the better. I can handle sinus infections, but the attacks that I have read on this website that others have suffered is really scary. I barely take a handful of aspirin a year, and taking chemo drugs and high does of prednisone when I feel ok seems a little crazy to me.

I live in the Philadelphia area, so if anyone can give me lung, ENT or rheumy recommendations, I would be most grateful. sorry for all the questions.

[Pete]

You may want to get an appointment with Dr Peter Merkel in Rheumatology at University of Pennsylvania. He's among the best in the world at treating Vasculitis.

Typical therapies for Vasculitis often include high dose prednisone along with an immunosuppressant.

The good news is that with proper care, you can have a near normal lifestyle. I'm 70, and I swim 1,000 yards twice a week.

Good luck!!

[John S]

Hi everyone, I'm so glad I found this website, although the more I read, the more scared I am. I, like so many on this forum, have led a clean life, no meat, no sweets, 2-3 hours of exercise a day for the last 30 years. My only issue was vertigo and sinus infections that lasted for weeks. Since January of 2016, I've had chronic sinusitis, and in December, had pneumonia, which I quickly recovered from, but shortly after finishing the antibiotics, I got a raging sinus infection and the flu. The pattern has been that a week after I finish the antibiotics and 5 pack prednisone, I start getting all the sinus infection symptoms back. Right now, my energy level is high, although I still have minor mucus discharge and tinnitus. I only usually missed 3 days a year of exercise, but in the last 3 months, I've missed an entire total of 1 month. Something isn't right. My family doc suggested I see an immunologist, and my pANCA was 1:3 and Lymphs were above normal. I went to an ENT to inquire about a nasal biopsy and he does sinus surgery along with it. I am opposed to doing that b/c he will put me on high doses of prednisone first and do the surgery, followed by more meds. If the nasal biop is negative, I will still need a lung biopsy. Is it possible to find a doc who will just do the nasal biopsy without the sinus surgery "package?"

Also, since i feel well right now, if the lung biopsy comes back positive, do I have to immediately go on those scary meds, even though I feel almost "normal?" I know my life is going to change, and not for the better. I can handle sinus infections, but the attacks that I have read on this website that others have suffered is really scary. I barely take a handful of aspirin a year, and taking chemo drugs and high does of prednisone when I feel ok seems a little crazy to me.

I live in the Philadelphia area, so if anyone can give me lung, ENT or rheumy recommendations, I would be most grateful. sorry for all the questions.


They might be able to see something with a CT scan of your lungs.

[lovetoswim]

Thank you, Pete. Will he be the person that would do the lung biopsy?

[lovetoswim]

Hi John, I had a chest CT. Are you saying they could see if I have Wegs?

[Pete]

Thank you, Pete. Will he be the person that would do the lung biopsy?

I don't know if he has surgical expertise. I know that he cares for several people on this forum, and that he's known as one of the best at treating Vasculitis.

Rheumatologists typically lead the treatment of Vasculitis. They work with other specialties as needed. In my case, I've seen ENT, pulmonologist, urologist, chiropractor, nephrologist, and neurologist as different issues have arisen.

I urge you to see a Vasculitis specialist ASAP. This disease can do a lot of damage in a hurry.

[lovetoswim]

I just realized he's the rheumatologist. I do need to find a lung doctor first, so I can get a definitive diagnosis, correct? Would Dr. Merkel treat me without having had a biopsy?

[lovetoswim]

Also, Pete, are you still taking medication? Were/are you able to exercise while on meds?

[Pete]

Also, Pete, are you still taking medication? Were/are you able to exercise while on meds?

I take 5 mg/day prednisone and 3/week bactrim. I also take rituximab as needed (annually for the past couple of years). I have also taken cyclophosphamide and methotrexate. My prednisone dosage has ranged as high as 60 mg/day. That gave me a ravenous appetite that helped me regain the 25 lbs I lost at disease onset plus about 20 more. I've lost 10 pounds of the overage, but I've plateaued at about 10 pounds over my pre-wegs weight. I've only been completely off prednisone for about four months, but disease symptoms reappeared. My last taper two years ago got me down to 3 mg/day before symptoms reappeared again, so I went back to 5 mg and have stayed there.

I was very sick at disease onset and had a major relapse about a year later. I resumed exercise very gradually and worked up to walking 3 miles 3-4 times a week. I resumed swimming a couple of years ago, but was off and on with it until sciatica from an old back issue kicked in, so I only walk once or twice a week for 1-2 miles. I now swim twice a week. I've been doing 5x200 yards on 5-minute cycles for the past three weeks. The ultimate goal is 1,500 yards non-stop in about 35-40 minutes. Golf season will be here soon (I hope), and I plan to play once or twice a week.

[John S]

Hi John, I had a chest CT. Are you saying they could see if I have Wegs?


They might. Ask your rheumatologist to look at the disc, not just the written report.

[lovetoswim]

Hi Pete, what were your symptoms when you were very sick at the onset? While on medication, did you resume exercise?

[lovetoswim]

Hi John, I don't have a doctor yet. Can I see a rheumy even if I haven't had a lung biopsy yet?

[John S]

Hi John, I don't have a doctor yet. Can I see a rheumy even if I haven't had a lung biopsy yet?

What Doctor have you seen? Has anyone run ANCA blood work, SED rate or other diagnostic tests? I guess that what kind of Doctor you can visit will depend on your insurance or ability to pay.
It is my understanding that Rheumatologists are the ones that diagnose and treat Wegener's Disease.

[Pete]

Hi Superswimmer!

At disease onset, it began with a middle ear infection that did not respond to the usual treatment, followed in short order with what started as a persistent cough that morphed into a productive cough with a little blood in the sputum. I also had a marked loss of energy and appetite. As things progressed, I began having night sweats (2-3 each night that thoroughly soaked my pajamas and bed sheets). I spent a week in the hospital at Ohio State where I was eventually diagnosed by a process of elimination. Thus treatment began with 150 mg/day of cyclophosphamide, 60 mg/day of prednisone, and bactrim 3 times weekly.

About a year in, my doctors switched my immunosuppressant to azathioprine (immuran). I had a very bad reaction to it resulting in pneumonia, hypotension (BP was 60/40 at one point), crushing fatigue, and persistent hiccups. This was much worse than disease onset as I pulled a ribcage muscle during one of my coughing spells. I went back to cyclophosphamide.

It was at about this time that I found this forum. Shortly after, I self-referred my wegs care to Dr Alexandra Villa Forte at Cleveland Clinic. She switched me to methotrexate (15-20 mg/week) and began a slow prednisone taper (took over a year to get off it). I was on methotrexate for three years before the side effect of post-treatment fatigue got too annoying. Thus, the switch to annual treatments with rituximab.

Between episodes, I was able to exercise. I started from being barely able to walk the hundred yards from my house to the nearest street corner, and I gradually progressed over a couple of months to being able to walk 3 miles in less than an hour. I did this indoors at the local YMCA and outdoors (weather permitting) 3-4 times a week.

I began swimming again shortly after disease onset, but the cool water and lung damage (since healed) caused me to have asthma and extreme shortness of breath. So, I didn't try to swim again until a couple of years ago. Again, it was a very gradual process to build endurance. The first time I swam again, I did 2x50 yards and was exhausted - it felt like I was swimming in jello. I've progressed to being able to swim 5x200 yards in 25-30 minutes twice a week. I'll continue to work toward my goal of 1,500 yards non-stop.

I've had to cut back on my walking because the sciatica from an old back injury has acted up. I walk 1-2 miles a couple of times a week. Chiropractic is helping, but I'm a better swimmer than walker.

This has been a long way of saying yes you can exercise while taking wegs meds. Do as much as you can, but pay attention to what your body tells you.

[lovetoswim]

John, I saw an infectious disease doctor who ran all autoimmune tests as well. Had a high pANCA and Lymph node levels. Would a rheumatologist be the next step or a lung doctor for a lung biopsy?

[annekat]

Welcome, lovetoswim. Your scenario sounds a lot like mine before my dx, with the string of sinus infections and eventually lung issues... the one thing missing is the massive refractory ear infection that was my first big symptom, but still not taken as a clue of what I had. It did take lung involvement and a CT scan to show things in the lungs that made a doc suspect WG. About the same time, I noticed my saddle nose and went in to the same ENT who'd been treating me since the ear infection, and got a nasal biopsy on the spot, which was positive for WG. So, yes, you can get that biopsy without also getting sinus surgery, and I don't understand your ENT'S viewpoint. What you need to know is whether you have WG. Of course, you might need a lung biopsy, anyway, since nasal ones are often not conclusive. I was lucky mine was, and it was not nearly as big a deal as a lung biopsy, so I feel it would be worth taking the chance on that first. Just go to a different ENT, and I think Pete's suggestion of Dr. Merkel as a rheumy is very good, since it sounds like you live near him, and he can suggest an ENT if you're having trouble finding one, as well as a pulmy. The fact that you say you feel pretty good is encouraging that if you have WG, it may not have progressed as much as some of ours at dx, but without treatment it will likely get worse to where you want the drugs that will help you get it under control. I just realized I hadn't read all the posts including Pete's excellent one describing his history, and how well he's recovered and able to do so many things, including lots of swimming, and the same could be true of you if indeed you have WG and are proactive in dealing with it. Don't be afraid of the drugs, the sooner you start on them the better, and the sooner you'll be able to get off the worst of them. Good luck and keep us posted.

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[John S]

You might want to see a whole team of Doctors, ENT, Pulmonary, Rheumatologist, Eye Doctor, your General Physician etc. depending on your symptoms

[Alias]

Nothing you've said indicates that a doctor has suggested a diagnosis of GPA? Certainly sounds like something is going on if you've had recurring symptoms for over a year, but has your doc said he suspects it? Standard workup would include chest CT, UA, ESR, CRP, in addition to checking for ANCA/ PR3.

Nasal biopsies are quite often not diagnostic; a Google search of GPA research will tell you that. Others have had different experiences, but I submitted to a nasal biopsy and it only worsened my situation. My biopsy was negative, even though I had positive C-ANCA/PR3, very high ESR and CRP and strong clinical indicators of GPA. In retrospect, I regret not following advice suggesting that the biopsy was overkill, as it caused a massive increase in scarring and inflammation. Especially if you are feeling well, not sure why you would pursue invasive procedures? I'd be particularly careful of the "bundling" of nasal biopsy with some kind of sinus surgery, if what you are looking for is only a diagnostic test. Do they even do lung biopsies when a chest CT is clear?

[annekat]

To add to the biopsy question, an experienced WG doc may be able to conclude a dx without one and begin treatment, based on bloodwork, symptoms, etc. In my case, the nasal biopsy was easy and successful, but everyone​'s case is different. And I forgot to mention an easy biopsy of my swollen parotid gland was done at the same time and probably contributed to the conclusion. A biopsy can come from anywhere that suspicious symptoms exist, such as in some cases, inflamed and bleeding gums. Seeing a good rheumy such as Dr. Merkel would be a good start, and he could help you decide from biopsy options and where to go for them. If he works out of a teaching hospital, of which I'm not sure, there could be others on the staff available​ to address the various parts of your possible dx and treatment. Best of luck.

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[lovetoswim]

Thanks, Annekat, for the support and information. I also had a parotid gland infection last year but it was resolved with antibiotics. I will continue to look for an ENT who will do a nasal bippsy in my area, and will contact Merkel tomorrow, but I fear there will be a very long wait. Trying to get an appt with a lung doctor at Penn resulted in an appointment in July! That's just for a initial consult.

This ENT who wanted to do the nasal surgery as well is not my regular ENT. My ENT that I love and trust, does not do nasal biopsies and recommended Penn or Jefferson.

Do you recall what they found in your chest CT that showed possible WG?

[lovetoswim]

Hi Alaskatom, I am not going back to that ENT with his, as you so aptly called it, "bundling" offer. No way am I going through any surgery I don't need. So my immunologist said my pANCA was high, but C-ANCA is normal and MPO is normal. He recommended a lung biopsy, as he felt the results pointed to WG. What is the ESR and CRP readings you were high in? I don't know what that means. My chest CT scan showed some scarring and patchy infiltrates--perhaps from the pneumonia I had in December. I don't know who to see first.:cool1:

[annekat]

Yes, what they could see in my chest CT scan was numerous small, scattered "cavitary lesions" that they surmised were the result of WG. The phenomenon may be different in other patients, with bigger lesions or nodules, containing granulomas, and they may be localized in certain areas instead of being scattered. The scan happened while under observation overnight at a hospital, so this was an overseeing doc who referred me back to my regular ones. My ANCA was not very high, but that's not unusual. I was coughing up a little blood, had the nasal and ear issues, headaches, eye redness, and trouble breathing. Within a couple days of getting home I noticed my saddle nose and had already been on the forum and knew what it was, plus my parotid gland was swollen. My pulmy was on vacation and I went right into my ENT office and got the two easy biopsies. The parotid may not have been infected but more full of WG activity, plus the doc wanted to rule out cancer, since it felt like a lump. Once the official dx came in I was treated by my pulmy as he felt competent to put me on the right meds and oversee my blood work and breathing function. I still saw my ENT to monitor my sinuses and ears. I should have had a rheumatologist but wasn't confident I could find one in my area with much WG experience. I did well on the treatment but think the pulmy had me on Cytoxan for longer than necessary. I've now been on methotrexate for a few years. My pulmy just retired, so I have found a rheumy in my community who seems to have some WG experience and seems nice, but I've only seen him for the initial consultation. I could go to Seattle for more experienced specialists, but I'm not​ much for traveling and also might have to wait awhile for an appointment. In your case, maybe you could find someone competent by continuing to ask around, and by contacting the Vasculitis Foundation for advice, or call Dr. Merkel's office regularly for possible cancellations​. And I've heard the same thing about getting new appointments with pulmy's and rheumies even here. I got a cancellation appt with the rheumy and haven't even tried to get a new pulmy appt yet, as my lungs aren't bad now. Maybe there is a local online group of Weggies in your area, perhaps on Facebook, who could help you with all this, too. And there are general vasculitis and Wegener's groups on FB, too, whom I or others can refer you to, where you can throw out a few questions. If you send me a private message I can hook you up with a couple. I wish you luck.

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[Alias]

Interesting, I also had parotid swelling, but it was not biopsied and subsided with standard treatment for GPA, i.e. prednisone and an immune suppressing drug.

Lovetoswim, the ESR and CRP tests are for non-specific inflammation markers. Positive results can mean many different things, but can point towards GPA in combination with other findings.

My initial symptoms were hearing loss, apparent ear infection, and nasal/sinus issues. I also had some vertigo issues for a while before I was diagnosed. My chest CT was clear, so there was no discussion of lung biopsy.

Generally, a rheumatologist would be the most appropriate specialist to address GPA issues, but an ENT or an internist could also direct you through at least the diagnostic stage.

[lovetoswim]

Thanks, Alaskatom. I've had hearing loss since the 1980's and it has worsened. I've also had a few violent vertigo attacks, and labrynthitis that lasted 8 months - it was horrible. Besides the parotid gland infection, I've had 2 eye infections, and what I thought was chronic sinusitis. What is so scary is that I've been to at least 4 ENT's and was sent home with dx of "hair follicles" for the swollen lymph nodes, or recommendation to have sinus surgery. After a year of this, no one has ever thought it could be anything more than chronic sinus issues.

I hope I can find a semi-competent rheumy at least until I can see Merkel.

[lovetoswim]

Hi, Annekat, I will pm you. Thanks so much for all your help. There is a nodule on my chest scan, as well as some "patchy infiltrates" whatever that is.
How is your "quality of life" on methotrexate?

[drz]

You may want to get an appointment with Dr Peter Merkel in Rheumatology at University of Pennsylvania. He's among the best in the world at treating Vasculitis.

Typical therapies for Vasculitis often include high dose prednisone along with an immunosuppressant.

The good news is that with proper care, you can have a near normal lifestyle. I'm 70, and I swim 1,000 yards twice a week.

Good luck!!

See Merkel ASAP!!!

[lovetoswim]

Seeing any doc in U of Penn is daunting. the next appointment is in July for a pulmonary doctor. With credentials like Merkel's, I can't imagine I could get in anytime soon until I have a definite dx, which i can only get from a lung biopsy.

[John S]

I contacted the Cleveland Clinic and saw Dr. Villa-Forte, a rheumatologist, within a week. She told me that she only works with Wegener's patients and always tries keeps her schedule open so that she can see new patients.

[lovetoswim]

My cousin passed along my "story" to her U of Penn gastro guy. he doesn't udnerstand why the doc who ran the blood work and dx'd me with possible Wegs didn't give me a lung doctor referral for the biopsy. I've hit brick walls at every turn. OTOH, this gastro doc claims pANCA can be high when there's infection going on. Is that possible? I have been quite ill over the last 12-18 months, but other than a lot of mucus, I've been working out like a stallion every day and feel really good. Could I still have Wegs?

[annekat]

See Merkel ASAP!!!
Try calling Merkel's office on a regular basis, like once a week, to ask about possible cancellations. This has worked for other forum members trying to see various doctors ASAP. Meanwhile, pursue other avenues if you can.

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[annekat]

My cousin passed along my "story" to her U of Penn gastro guy. he doesn't udnerstand why the doc who ran the blood work and dx'd me with possible Wegs didn't give me a lung doctor referral for the biopsy. I've hit brick walls at every turn. OTOH, this gastro doc claims pANCA can be high when there's infection going on. Is that possible? I have been quite ill over the last 12-18 months, but other than a lot of mucus, I've been working out like a stallion every day and feel really good. Could I still have Wegs?
I wonder if that doc meant inflammatory markers such as SED rate and CRAP can be high when there is an infection. I think ANCA is more specific to certain forms of vasculitis, including WG. I could be wrong.

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[annekat]

Hi, Annekat, I will pm you. Thanks so much for all your help. There is a nodule on my chest scan, as well as some "patchy infiltrates" whatever that is.
How is your "quality of life" on methotrexate?
I will look for your PM. I don't know much about the various manifestations of WG in the lungs, just that mine were cavitary lesions, and now they say there is a benign nodule which likely grew out of one of these lesions, which they think is filled with fluid and air, and harmless. But a good reason to find a new pulmy, just in case, since mine retired. As for methotrexate, I usually feel ok on it and it hasn't caused any liver issues in my blood work. I did feel yucky on it at first, and for awhile, the day I took it, and that can still happen if I'm run down or low on sleep. It helps to split the dose, like take half at night and half the next day.

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[Alias]

MTX is pretty widely prescribed and well tolerated by many. It is effective in inducing remission of GPA for a lot of patients and is not expensive. It's also used by a lot of RA patients. I've been taking it for over three years with no side effects other than occasional mild nausea the day after using it (I take mine via injection once weekly) and minor elevation of liver enzymes shown in labs once in a while.

It seems from what you've said though that you have not been definitively diagnosed, and it's confusing to me that you say you have been very ill but that you feel great. Sounds like you have something chronic and/or recurring that is mysterious, and that GPA has only been raised as a possibility?

[lovetoswim]

Yes, Alaskatom, I do feel really strong now - finally all the mucus (the last surviving symptom) is gone. But, it's been a year and a half of dozens of visits to ENTs, at least 6 prescripts for antibiotics and 5 pack prednisone. The worst was in December when I got pneumonia, then the flu and a sinus infection that my ENT described as "raging." He did a culture and 4 bacteria were found. My primary recommended an immune doc. I had b/w done that showed high pANCA and Lymph node readings. That doc thought it was Wegerens and told me to get a lung biopsy. I don't even know if the proper tests were run. And from the research I've done on line, I can't find anything other than vasculitis as a cause of high pANCA. Do you know if pANCA could be high as a result of an infection?

[Mrs.M]

Hello. If I understand you correctly, you haven't been diagnosed yet. If it turns out that you have WG, it is something that you can live with. My husband has had it for twelve years now. He goes to the gym every day - he does cardio and weight training. So, please don't think that you're life will go downhill from here. Once you get your team together and get on the right meds, you may find that you will be able to live your life and do it well. Best of luck to you!

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[Rachel_Elise]

I would highly suggest seeing a Rheumatologist first. They will be your go to doc and help your coordinate any biopsies/treatments that you need. A Rheumatologist should see you with out a diagnosis, and will probably want to run a battery of other blood tests and such to get a feel for where your at. As for the sinus surgery - there should be no reason for you to have the sinus surgery as well as a biopsy. I got talked into a sinus surgery at one point (when I was not under proper care by a Rheumatologist) and it didn't help at all with my chronic sinus infections. A Rheumatologist that knows about WG can help you negotiate that though.

As for all of the meds - yes they can seem a little scary, but they are important in keeping you healthy. But what you will end up taking, all depends on how the disease is effecting your body. If all you have is sinus involvement, they may try treating you with only low doses of prednisone. This will help with the sinus infections, and usually helps me a ton with my fatigue. At low doses you may notice some minor side effects, but it's really not bad. The other types of drugs may be needed if the disease is more advanced (which it doesn't sound like it is), but even if they are needed, the side effects are usually minimal.

You can still lead a normal life, and exercise as much as you feel able to. Exercise is good for you no matter what, and my doc encourages me to do as much of it as I can without exhausting myself or irritating my joint pain too much.

Stay calm, and find a good doc that can help you through all of this. I wish you the best of luck!