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Is infusion therapy necessary to achieve remission in egpa

I am wondering about achieving remission. I do not think that I have ever actually been in remission. My new rhumatologist/vasculitis specialist, who I have only seen for the past month, believes I have ANCA negative egpa. Getting a diagnosis has been very difficult and I have seen many doctors along the way but it has, and still is, very confusing. I read about patients having infusions to achieve remission. My understanding is that six to twelve sessions are standard and then with good luck disease activity may disappear for years. Am I understanding that correctly? I don't know when my egpa started ....years of breathing , sinus, and ear problems and then brain damage with eosinophils of 17,000 in 2016. High dose oral steroids....misdiagnoses....etc etc....until today. Currently I am on 30 mg prednizone ...hopefully only for five days and then back down to 10 if I can stop the rattle in my chest...and methotrexate one a week which I started a month ago. I want to trust the new doctor but I don't think I feel myself getting better. Thoughts?

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Hi,

I am far from an expert, but my understanding is that in severe EGPA ( that has affected kidneys, heart, brain for example) infusions are required to control the illness.

If they want to control the illness fast, cyclophosphamide will be used as Rituximab needs some time to work.

I had 6 infusions over 4 months ( I have GPA) and then was put on azathioprine. I am in medical remission for more than 3 years without a flare.

Be very careful when reducing steroids, tapering too fast can cause a relapse.

Good luck with the treatment.

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Thank you for the explanation... can you further explain what are some of the drugs ,including the ones u mentioned, used to control egpa. It is confusing because I think they must go by different names

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Hi Cathy, your path sound familiar to mine. I think I've only been in the eosinophilic phase for a couple of years, and they have been unable to keep the eosinophils down for more that a couple of weeks, despite high dose pred. for a years (30-50mg/day). Have now switched to Triamcinolone (80mg) every 8 weeks, which has been more successful, along with Azathioprine. The new plan seems to be a new Asthma biologic - reslizumab IV to control the eosinophils. This is delivered as an infusion. I tried Mepo earlier this year but randomly lost a lot of lung function.

There seems to be so many different opinions flying around that its difficult to figure out which option is the right one....

I hope you find something that helps soon

Elaine

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Hi Elaine

Yes I read your posting of a few months ago and our paths are very similar. What a silly waste of time, energy, and life all this messing around with doctors has become. I have been gathering information from people on this site for about a year now and it sure seems to me that we could get fast and efficient care if we could put the proven good egpa doctors from around the globe into one room and ask them to figure out treatment for us. Like you, I have been struggling with seemingly unrelated symptoms and illness for years. I can understand my GP not seeing the link and guessing at the bigger picture but once my eosinophils hit 17,000 and my brain shut off I am guessing that someone, other than me, could have put the pieces together. But no. Sadly, I think the medical system in Canada is not designed to allow patients to be partners in their medical plan. The whole thing is more sustainable if the patients quietly do as they are told even though we often know in our hearts that it is grossly incorrect. Sorry I am rambling again. How many people must get wrong treatment and simply fade away when they could have been helped? This just isn't right. I want to do something to help

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Hi -

In order to help, could you clarify what exactly EGPA is?

I have Wegeners,

Hofmann

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It is an eosinophilia disorder. it seems very hard to get a diagnosis for us people who are ANCA negative. If the blood does not have antibodies to prove the diagnosis then even though ONLY 40% of patients actually do have such antibodies doctors do not believe that egpa is possible. Which is so strange because, according to my research, among experts there is no question that most patients are never ANCA positive. Egpa used to be called churg Strauss. It is a form of vasculitis although I think experts may be looking at ANCA positive and ANCA negative egpa as perhaps two different things with vasculitis being more prevalent in the ANCA positive patients. I am just learning . I really had no choice since I ended up in hospital with crazy high eosinophils and no diagnosis except.... idiopathetic hypereosinophilic syndrome....which I learned after months of reading, simply means....your eosinophils are super elevated and we have no idea why. Not much of a diagnosis and for me the worst thing was that once that label was pinned onto me I could forget about getting any medical help. I was told there is no centre of excellence for idiopathetic blah blah anywhere in North America and no specialists AND the only treatment is steroids. They wanted to start me on a chemo drug as they said idiopathetic blah blah probably was actually cancer but I refused and tried to find another option.

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Very interesting thanks Cathy.

I too have questioned my diagnosis and as a retired GP have convinced my neurologist (my "wegeners/GPA" has now become neurological with a question-mark over original diagnosis!) to go down the route of TB (yes, Tuberculosis) as the route cause of my symptoms and MRI brain findings (lots of unexplained white spots). I have significant neuro symptoms. My story fits TB, and I am waiting to see Dr. Hadi Manji in The National Institute for Neurology and Neurosurgery, London who is a neurologist with special interest in infectious diseases. Unless he convinces me otherwise, I plan to go onto a treatment regime for TB which my own neurologist in Northern Ireland has agreed to. I was on methotrexate and rituximab but stopped both as was getting worse. I was seriously unwell last year and high dose prednisolone saved me (I increased the dose myself to 60mg daily).

Essentially, nobody knows for sure what my diagnosis is but the BIG TWIST in my story is my brother, nine years younger than me, has developed symptoms 70% similar to mine over the last 9 months and has been given a diagnosis of AAG (autoimmune autonomic ganglionopathy). His consultant is very open to being wrong - I saw her myself to gauge her thoughts - and she is kindly writing to Hadi Manji in London to let him know my brother's situation in advance of me going to see Hadi for admission for a week of tests. My brother James has improved on IV immunoglobulin, rest, a beta-blocker and Venlafaxine. His only positive test was a Tilt Table Test. The only existing accepted blood test for AAG was NEGATIVE!

This is what we are ALL up against and why we must keep questioning and also consider going down alternative/crazy routes as I am doing with my TB theory.

Hofmann

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I am humbled to read that you are a GP and are still left clinging to the side of the lifeboat with the masses. I knew nothing about any of this before sept 2016 when my eosinophils went to 17,000. I had to do my own research to get any answers but I assumed I just had bad luck and that most patients don't need to do any searching because the medical specialists would surely have all the answers. I was so wrong.

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You know you have me thinking again.....I had lots of brain issues when I ended up in hospital in 2016. My eosinophils were 17,000 and I was not really even conscious. After a few days I was started on prednizone and the count went down but I was left with lots of "white spots" on brain scans which I don't know anything about except that I think it causes stroke like damage. The doctors told me egpa never affects the brain so for a long time I was left with idiopathetic whatever so I kept searching. I know that something is causing my eosinophils to keep going up and I need to find out what it is. I tried to get a consult with infectious disease but couldn't so I read what I could about assorted infections. I think I started getting really sick about the same time as we bought a cabin in the tick infested wilderness. Although Canadian doctors don't seem to agree, some believe that Lyme and its coinfections can create horrible debilitating infections. So....I travelled to the other end of the country where I could pay out of pocket for some Lyme testing and it did come back positive. There is not much really to do with that knowledge as it isn't even recognized as a real thing where I live but who knows? Maybe in time we will find that some of these things are triggered by assorted infections....and then maybe we can just treat the infection and carry on

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yeah, all my tests negative for infection including for Lyme disease and TB.

however my STORY fits TB as a possible cause and the problem with all these so-called "auto-immune" diseases is that effectively the symptoms and signs are being treated with very toxic drugs WITHOUT the cause being known so the patient ends up in a fingers crossed it all works out situation. In my experience as a doctor, most times it doesn't!!

H

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I am just wondering if there is any treatment for tb other than long term antibiotics. I hear that lots of people with egpa, myself included, have brain issues, lesions, white flares, and yet I hear again and again that it doesn't affect the brain.....usually. Your theory of your illness being associated with a brain infection, tb, is an idea I haven't heard before. What if it actually is related? If "infection" causes this disease then steroids and immunosuppressants can't cure us....can they? I told my new doctor my thoughts on a possible Lyme infection connection, since I had a positive Lyme test, but he wasn't interested. I am so curious to hear how your treatment goes!

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Will keep you informed,Cathy. My plan is to allow the London consultant to come to his own conclusions and if he has no new ideas than I will go on a TB treatment regime for nine months at least which my own neurologist in Northern Ireland has agreed to. He is very open minded fortunately and when I told him about my TB theory and how I had increased my steroids myself to very high dose (55mg daily last year) he was interested and responded immediately that TB is STEROID-RESPONSIVE! I still have significant symptoms daily so believe I do need more than just steroids but preferably antibiotics rather than another blind trial of a different, toxic chemotherapy drug!

Did you ever get treatment for Lyme disease??? If yes, what with, for how long and what was your response??

Henry

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Hi Cathy , I was diagnosed with EGPA this time last year after collapsing at home a few days after a combination of symptoms became very evident ( Sinus problems for over a year , excruciating random joint pains, skin nodules , a sudden rash , stomach cramps, chest issues) I was taken to hospital and on release started on Cyclophosphamide infusions . I had 5 out of the 6 planned infusions over 4 months before being moved onto 20 mg Methotrexate that I take weekly like you . I am also down to 10mg of prednisolone daily from the 60 I was on before .

After a year the worst symptoms are under control with some sporadic mini flare ups but I can’t get back to ‘normal’ yet and every day is unpredictable. I am now being considered for Rituximab as well as methotrexate but they want to check my ANCA levels again . I was ANCA positive but you are right only 40% of EGPA suffers are ANCA positive so it’s not the definitive test & it appears none of the indicators alone are definitive as my eosinophils were nowhere near your levels .

So in answer to your question infusions are used it depends on the severity at the time and also the guidance for the country you are in I believe . For example I had a second opinion for my case through an insurance policy and the specialist in the U.S recommended Methotrexate for 12 months whereas as the British guidance I have read and as per my consultants view is 5 years so it does depend on where you are as well it seems

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I recently attended the VUK group meeting, which incidentally highlighted the mainstream Rheum's focus on the large artery vasculitis/"ANCA" as the main. That's the star of the show. Some patients commented, this would actually exclude others, who are suffering just as bad (and possibly die, prematurely), but there was no answer to that question. It just seems that's the way it is and there you have it.

Antibody obsession is often seen in other autoimmune diseases. I guess clinicians want to see the "evidence" of the disease presence, "but" (as always, "but") there are ANCA positive patients, who have no symptoms. Shall we say, maybe, some change is needed on this aspect for V patients?

Without meaning to sound "morbid" unnecessarily, I wondered the mortality rate amongst V patients who had a single or multiple organ compromise and what was the cause of their death. I felt compelled to ask.

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And?

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Sorry I misunderstood your last question. Did you say you asked about mortality rates at the conference or are you asking the group

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Hi Cathy

After 11 years of attending doctors witch increasing symptoms of allergies, rhinitis, sinusitis, asthma, loss of smell,taste and hearing I was in so much pain in my arms and legs that I presented myself at QEUH A&E dept. After several days I was diagnosed with EGPA at the age of 55. Many of my organs had been damaged including liver, spleen, left eye, nerves and muscles in my legs and right arm and hand. Above all, my heart was damaged and I had to have a defibrillator fitted to my heart. The day after it was fitted I was allowed out of hospital - 5 weeks after going in. I have been a very fit man all of my life and played tennis 3-4 times and golf 1-2 times per week as well as going to gym. I found that I was very tired but keen to regain my fitness. On pred - originally 80mg now down to 5mg and 125mg of azothioprine after 6 infusions of cyclophosphamide that put me in remission within 6 months of being diagnosed. I was off work for 6 months recuperating but this allowed me to walk and go to the gym regularly and rest appropriately. I had an 8 week phased return to work and have been working full time for 9 months now. I can play tennis and golf but still can’t hold the racquet or clubs properly with my right hand. I get tired more than I used to and don’t push myself to do as much cardio exercise as as I did previously. The nerves and muscles in my right hand and arm were damaged to an extent that I could not bend my thumb and 2 fingers. After 17 months there is a massive improvement. Only now do I feel that I will get back to somewhere near my previous fitness levels despite being very positive throughout. I have nothing but praise for the medical staff that have treated me since my diagnosis. I guess the main thing is to get your diagnosis of EGPA confirmed then hopefully the treatment of infusions sorts you out as well as it has worked for me.

Harry

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Wow it is nice to hear a positive story.

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Good morning Cathywp

I empathise with you and so many people on here. This dreadful disease can be relentless and little wonder you have questions and want answers. I cannot give you ... and goodness knows it is difficult enough for the professionals and our medical advisors, due to so little being known or understood about this. I pay little attention now ( diagnosed with cryoglobumic vasculitis in 2017). by blood counts and all the technical issues .... I am led by how I FEEL not what my bloods say !! . The only advice I can offer ( purely as it works for me ) is to keep daily notes of HOW YOU FEEL and ALL symptoms .you are having .. this helps to increase the good days ... and is hugely valuable to your consultant (and gp as many of them have never treated patients like us ). What you eat and did each day can have great effect on how you feel . Medication often confuses us and it’s difficult to seperate “symptoms “ of the disease and side effects of the meds!! Develop a strong relationship with gp and consultant and ASK as many questions as you have !! The diseases we have are rare and s often unique to ourselves !! Keep strong , as positive as you can ... it’s not easy at times ... and any improvement can take time ... I so hope you get to a more comfortable place soon .

Kind regards

Alan

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Thank you so much for the kind words. My husband also keeps telling mr to write it all down as it seems since my hit to the head I have a difficult time remembering that I do actually have both good and bad days....I honestly can't remember what I felt like yesterday or last week.

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Hi Cathy,

I believe that we chatted almost a year ago. I too am ANCA negative and was diagnosed with EGPA in 2006. I also live in Canada, but am fortunate to have lived in Toronto, where there are specialists in vasculitis. I have been treated by Dr. Rachel Shupak for that entire time. My sense is that in the past few years there has been some change in the treatment of the disease. 13 years ago, there was no discussion of infusions. I was treated with high doses of prednisone, starting at 100 mg, and a very slow tapering to 60 mg [taking 4 months), and then methotrexate was added. That gave me liver failure so I was switched to cellcept [also known as mycophenalate] along with gradually tapering prednisone. It took 2 years to get off the pred. For 8 years I stayed on mycophenalate.

So the big development in the past year is that I've entirely tapered off the mycophenalate. A month later, I had a minor episode of vision loss on the outside aspect of my right eye. My doctor wondered about MS, so I had a brain scan. It showed 6 foci of ischemic change [white spots like you had]. My rheumatologist did not think those were evidence of EGPA significance, however, I've seen a couple of studies that found "multiple ischemic foci". Nevertheless, I have been referred to a neurologist.

I'm also scheduled for a consult with an orthopedic surgeon for a hip replacement. I did get degeneration in my joints from the heavy prednisone. So it all sounds terrible, but I'm doing ok. I am lucky to have been diagnosed early. Perhaps now there would have been different treatments, but this is where I am. I can manage difficult and complex work, but it takes longer. I can walk, but it takes longer. etc. etc. Yesterday, I played water volleyball for two hours, so there can be health, but it's different.

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Yes Karen we did converse a year ago and I was wondering how you are doing. Sorry to hear about the vision issue! Hopefully it will just settle down and leave no permanent problem. Is your normal vision back now? Karen I have been wondering about what the heck can be the root cause of all this insanity. In searching for a diagnosis I went to Vancouver and had myself tested for Lyme disease. They did test me for that previously in Manitoba and at the mayo clinic but they used THE approved test and I have read that test is very unreliable and often gives false negatives. I felt my symptoms really matched Lyme so I had a different test done in Vancouver. For about $1,000 they send your blood to a German lab and do a different type of Lyme test. That came back positive. The naturopath felt I did not have an active Lyme infection at the time but rather that I had likely contracted the infection but had been able to fight it off. The reason I felt so strongly about Lyme was that I started to have weird symptoms that are not anything like what I hear egpa patients describing. But I have the egpa stuff also and it all started at the same time....I know Ontario is a hot spot for weird and wonderful Canadian ticks. I have a cabin in the woods near kenora which is the best place ever to catch Lyme and coinfections. Lyme is a neurological disease from what I read. It can mess up your vision. One morning I was sitting up in bed, watching the door , when the verticle hold went out of control in my vision...it was like a 1960s tv with the picture rolling! That lasted a minute and I thought well I'm really screwed now....but then it stopped and I forgot about it until I was researching again and read about it in a persons Lyme disease story. Other weird symptoms are hearing a roaring motor in my ears all night, feeling like the bed is shaking, freezing in my lips and mouth like I was at the dentist, severe muscle cramps everywhere, leg weakness, etc and all these symptoms were well before I started on any treatment for anything so I can't blame steroids. It all just makes me wonder if our poor bodies are producing eosinophils in an attempt to rid us of some kind of lasting bug.....

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EGPA is extremely rare and seems to affect men and women equally. Around 11-13 people per million are diagnosed with EGPA. It is essential you are seen by doctors who have experience and knowledge of treating EGPA. There are Multidisciplinary centres in London, Cambridge, Manchester and Birmingham. There is also a doctor in Portsmouth who has a special interest in EGPA. This page on the VUK website explains about EGPA and the possible different treatments. vasculitis.org.uk/about-vas...

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Thank you....it sure looks like you have more treatment centres in the U.K. Than we have here in canada

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There are more Multidisciplinary Centres for treating and managing vasculitis now in the U.K. Birmingham QE and Addenbrookes Hospital have been around for many years but the newer centres such as the Kellgren Centre and the Hammersmith have only emerged in the last 10 years.

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