Vasculitis (Churg Strauss) As My Cause For Pots

[RichGotsPots]

Many of you know me for two year now and it has been a long down hill battle against POTS, IST and Orthostatic Hypotension. I diagnosed all this on my own then went to 2 different specialist who confirmed it. It took me about a year to finally get a skin biopsy for autonomic neuropathy which showed I only had 19% of my sweat gland autonomic nerves. Then 2 months ago I finally got confirmed for Sjogren's Syndrome and autoimmune illness. But after talking to so many Sjogren's people I found that Sjogren's just didn't help to describe my breathing problems.

My Sjogren's Rheumatologist happened to do a detailed White Blood Cell Count for Neutrophils, Baso Phils and Eosinophils. My Eosinophils were 3-4x as high as normal and my Sjogren's specialist didn't think that was remarkable so I kinda of forgot about it.

Well two things came together for me. The first one was I was researching a giant chapter in a book that lists each and every cause of neuropathy broken out by types of neuropathy. The autonomic section is brief and does not mention vasculitis. But I was just browsing the other sections and I came accross Churg Strauss and it said high Eosinophils were a classic sign. BINGO!!! So I double check my Eosinophil labs and looked up the normal values and mine were sky high. Well that's one symptom in the criteria. The other thing that came together was I openned an old laptop I had and I had old documents on it I used to bring to doctors when this first started and on one of the documents was a list of breathing conditions that could be causing my problems and what do you know Churg Strauss was at the top of my list, hut my pulmonologist back then blew off everything on the list.

Okay so here is the criteria for Churg Strauss all put together. There are 6 criteria and 4 are needed for a diagnosis, just like with Sjogren's. I had 4 out of six in Sjogren's. Now I already have 4 in Churg Strauss.

1. Late on-set asthma that can become uncomtrolled with to prednisone or pulmonary infiltrates-got it at 27 and its not controlled anymore like it was when I was 27

2. Sinus problems- in 20008-2009 my sinuses were so bad I couldn't breath through my nose at all, couldn't taste anything or smell anything so I had surgery to have my sinus polyps removed, but still gives me trouble.

3. High blood Eosinophils- as mentioned above

4. Peripheral Neuropathy- I have autonomic Peripheral Neuropathy, its not typical so I will have to see if the specialists accept this one

5. High Eosinophils in any biopsied tissue- never had this before if they would have known in 2009 maybe they could have biopsied the polyps

6. Necrosis of any organ- this usually is life threatening and the final stage of the illness

The illness usually starts in stages. 1. Asthma and sinus stage. 2. high eosinophil stage 3. Necrosis stage

There are other issues associate with this illness. It is an ANCA antibody vasculitis like Wegners but only half the people have positive ANCa in Churg Strauss. There can be inflammation in the heart, lungs, kidneys and Gastro system. The most famous person who had it is Ben Watt from the band Everything but the Girl. They caught it late for him and it destroyed 80% of his colon but he is alive and way better now and alive with 3 new kids after 10 years or more.

It just so happened one of the top Vasculitis doctors moved from Boston to Philly only 90 min away 3 months ago. I am going to see a dr he hired in 2 weeks because he didn't start taking new patients yet. Wish me luck! I'm hoping this gets me IVIG and I found a new drug that has little side effects for treating it before they used high dose steriods and a chemo drug in combo and then mexatrexate after that to maintain remission. This new drug is not as toxic and just got orphan status approval by the FDA.

Your friend,

Rich

[Tachy Phlegming]

It will be interesting to see what they say.

I think you're the only person on here who has ever brought up the topic of Wegener's granulomatosis (and I don't know that any of us really understood why you looked for or found anything like that at the time). If you thought what you had was maybe that too, well, maybe what you have is in fact Churg Strauss Syndrome.

http://www.cssassociation.org/patient-stories-reader/items/solving-the-mystery-of-my-wifes-illness.html

(if you haven't already read the patient stories on that site, read a bunch of them).

Have you ever tried Prednisone?

[corina]

I admire your perseverance Rich and hope you will finally get answers and hopefully treatment that gets your life back on track! Thanks for sharing!

[looneymom]

Happy for you Rich. Being Persistent Pays. Sounds like you will soon have a new treatment plan soon. Keep us posted.

Rachel

[Katybug]

Glad you're getting somewhere with dx. What is the orphan drug you found? Hope they let you start a treatment regiment soon...it's been way too long of a wait.

[Guest Alex]

Rich,

good to hear from you.

I hope your perseverance will pay off soon. With this new doctor on board it sounds like you'll get the right diagnosis and you'll be able to start the treatment you need. Fingers crossed that you get approved for IVIG (3rd time's the charm they say... )

Keep us posted.

I'm rooting for you.

Alex

[sue1234]

Good luck, and keep us posted!!

[Chaos]

You certainly have worked diligently with your research. Funny how the things that frequently come up first, keep coming up time and again.

Hope you get your answers!

Best wishes!

[spinner]

Rich, you inspire us to all

1. be unendingly persistent in our research, diaries, etc

2. more of less become "our own doctors" in the sense that we are more likely to ultimately diagnose ourselves before they do since the battery of tests to determine an unending list of chronic illnesses is inumerable.

Who knows who can be helped by something someone here posts?

Ive been through rheumatology and been given the old "fibromyalgia" junk diagnosis that they give to

so many people who DO NOT have that.

My primary problems are breathing, heat, weakness. I dont have the traditional POTS. I tend toward sinus bradycardia, slowheartbeat. Cleveland Clinic at least found partial anhidrosis--sweating issues in a part of my body. Ive ruled out mast cell via bone marrow biopsy.

And supposedly they ruled of Sjogrens.

But you've given me something new to research. I certainly fit into the "late onset" aspects of these problems. Dairy was almost an

overnight flare.

Edited October 3, 2013 by corina

[badhbt]

Wow, I wonder how many man hours you have in research! That is awesome though....give yourself a pat on the back. I hope this answer gives you relief from your symptoms.

[spinner]

Another thing about all this is that-----many of us here dont have traditional POTS. We may have dysautonomia and related

chronic diseases.

Ive never had uncontrolled heartbeat/BP upon standing, but only upon exertion. But i have EVERY other symptom, confirmed sweating issues, the entire lot of problems.

Ive even got sleep apnea, and have been using klonopin for 2.5 years like so many people here. Seems to be the only

drug that works in the various nervous systems during a flare. And like Rich im constantly doing research looking for

these correlations that will lead to a final dx, and it would be nice to discover a cause too---although unlikely

[looneymom]

Hi Rich,

Just wanted to ask you if you have had a Paraneoplastic Autoantibody test done through MAYO. You mentioned Peripheral neuropathy. This test measures 15 different antibodies. The CRMP 5 IgG S is an antibody that identifies Peripheral neuropathy. This test panel is going to be ran on my son. I have started looking at the other antibodies in this test to see what they identify. Thought you might want to know about this test.

Rachel

[RichGotsPots]

Hey Tachy Phlegm, my asthma used to be controlled with very high doses of inhaled steroids, this time around when I ended up in the hospital in 2011 they gave me inhaled steroids and IV Prednisone which helped maybe 15% but then out of the hospital they told me to taper. But I suspected either autoimmune or neuromuscular back then and made this pulmonologist give me a bunch of prednisone. i took about 50mg for about 2 months but it didn't help. For Churg Strauss a lot of people become resistant to steroids alone. Also since I have autonomic neuropathy I really think steroid don't work like they should. I read a Cleveland clinic study that said that was 100% true for Sarcoidosis patients, which is also an autoimmune disease. And in that study they said IVIG helps 66% have a complete recovery.

[RichGotsPots]

Corina, Thank you!

looney/Rachel, Thank you! To answer your q. About paraneoplastic panel from Mayo, I have not taken it but I have known about it 2 years now, but no doctor around here wants to order it which is so wrong. I think it needs to be standard protocol. I believe 80% of all POTS is autoimmune related and that it will become a rheumatologist disease eventually and that all pots wacky symptoms are do 100% to autonomic neuropathy. We have different symptoms depending on the type of nerve damage. POTS will be like a subset of Guillane Barre. Also I know about 30 people who had that panel done and found antibodies and got IVIG from it. Some had AAG and some had LEMS or other autoimmune illnesses. Eventually they will had twice as many antibodies on that panel as they keep finding more and more.

KatyBug/Katie- thank you! Kthe orphan drug is Mepolizumab (proposed trade name Bosatria) is a humanized monoclonal antibody that recognizes interleukin-5 (IL-5), and is used to treat certain kinds of asthma and white blood cell diseases.

Alex- Thank you! i will for sure update everyone after my appointment in 2 weeks.

Sue- thank you!

Chaos- thanks! I promise even if I recover completely I will keep up my research for the sake of the whole group

badhbt- probably a few hundred hour of research in the last 2 years. At times I got burned out I couldnt look at studies for a couple of months lol for someone who would never do this in his whole life I really had to learn to read medical termonology and read between the lines. A lot of conclusions in studies are theory and I don't always draw the same conclusion as the researcher. Only person who I think can relate is Rama. He has been at this many many years and he also doesn't have a medical background. When I first started talking with him, like many, I thought he was a doctor or scientist lol I like to be the driver of my own destiny but illness takes that away from us, so over the years I found that knowledge and research gives me a little bit of that back.

Spinner- Thank you! I never thought about having to become my own doctor but this journey certainly forced me down that path and now with all this newfound medical knowledge I try to put it to good use for others. I must have looked into over 200 illness very deeply and maybe another 200 briefly. I was never interested in science so naturally I wasn't inclined towards medicine but all that's changed because our medical sysmtem is seriously flawed on so many levels. If I get healed I am thinking of going for a masters in public health to change the whole system. I used to be a journalist at one point in my career and maybe I will write about about the mess we call a medical industry or else maybe a few articles at the least. I have seen nealy 35 doctors in the last 2 years and about ten were well intentioned but only about 3 were well informed. There is no one size fits all in medicine but sadly thats how they treat us. Dysautonomia is a great example of how we are all different in so many ways. My feeling is that you have small fiber autonomic neuropathy and not Fibro at all.

I will for sure update everyone after the visit, thanks everyone for all your well wishes!

Rich

[Tachy Phlegming]

What a mess that steroids don't work for you. It would have made your life a lot easier (probably actually even without knowing what you had).

Oh, and one more thing. I assume you never tried Singulair, and particularly not while you were on Prednisone. There are a number of studies which show people "get" Churg Strauss while on both -- and if that happened, it might be fairly easy to have CSS diagnosed.

[RichGotsPots]

Tachy- back in 2006 when they diagnosed me with asthma they gave me Singulair for 3 months and I actually did get better but they stopped it since they didnt think it added anything. I did read the singulair and churg strauss warning thats probably the first time I heard of Churg, but I recently watched the Vasculities Symposium videos and on there the Mayo Dr. says that he doesn't think there is a connection at all. He says that it's just a coincidence that so Churg people with asthma don't know they have churg and then they are given singulair and later they find out they have Churg. Who knows...

[Tachy Phlegming]

Were you on Prednisone at all when you took Singulair in 2006?

While I too might bet that the combination doesn't cause CSS and the story is exactly what you wrote above, if you indeed took both at the same time, doctors will --because of those studies (even if they're wrong) -- be required to take the idea that you probably have that disease very seriously.

[RichGotsPots]

Tachy- I took high doses of inhaled steroids at the same time not in pill form like with prednisone.

Luckily I think I am going to a place where they will take CSS very seriously. I wish I was seeing the main dr there so we will see what happens. I go in one week.

[Legendlily]

Hi can you please contact me I am having similar symptoms and need advice solivo5178@aol.com