Hi Cathy some of your questions are too technical for me but I have EGPA or churg Strauss as it used to be known. It is a type of Vasculitis. Mine was diagnosed through high numbers of esinophils in my blood I understand. I suffered severe foot drop & damage to my peripheral nerves so they did take a biopsy in my lower leg. However they didn’t get what they needed. They also talked about taking a lung biopsy but decided that would be too invasive. I had lung involvement & have been left with asthma. I had never taken steroids before being diagnosed - late onset of asthma in my 40s was a sign of my disease. I had suffered from bad allergies to food, wine dust - usually sneezing fits & it was a blood allergy test in the hospital that picked up something was wrong. I did try taking montelukast prescribed by my GP to help with allergies, but not long term. I’ve never really gone into whetherits ANCA positive or negative - never needed to just trust my consultant to monitor my blood properly. Yes the diseases causes the blood vessels to become inflamed & swell which stops the flow of blood to vital organs causing damage - for me it was to my lower leg and so I have nerve damage & although this has got better as I can walk now - my feet & walking are not perfect. Hope that helps -
Hi Cathy it’s been about 6 years since I was diagnosed. I’m doing pretty well thank you. I have quite a demanding job so I have reduced my hours to 3 days a week - I still have lots of medical appointments. I no longer take prednisolone except when I get chest infections which I’m prone to about 2 or 3 times a year. I take 150mg of aziathioprine which is being monitored by Manchester hospital consultants as the moment as they found I had too much of the drug in my system and was at risk of damaging my liver, I’m under an endocrine consultant as taking high doses of prednisolone has affected by body’s ability to produce natural steroids and so I take a low dose of hydrocortisone. I take 20mg of amitryptiline for nerve pain each night and I’m under a respiratory consultant & on a high dose of symbiotic which I can’t reduce v much without repercussions.
For me foot problems have been the greatest long term damage that the disease has caused as they never properly recovered from severe foot drop. The nerves still misfire, I have loss of sensation and rather misshapen feet which affects my walking and balance. I have looked into surgery which isn’t a great option, spent a fortune on supportive trainers to try & accommodate innersoles & spent hours with the hospital’s mobility team trying to come up with comfortable options.
Im lucky as I can lead a pretty full and active life & I’m a gym addict but things have changed for me - lots of time in medical appointments, no more long walks in the countryside, when I get ill it is more problematic with altering medication & recovery & pacing myself - I take more power naps & watch many of my favourite programmes twice as I fall asleep a lot in front of the telly.
It has been a case of trying to be patient with the disease, learning to tolerate and experiment to some extent with medication making adjustments to my life and knowing how far to push myself to achieve a balance. Best wishes and I hope things get better for you too.
It sure is interesting to hear other people's stories! I find I compare my own story to others and I become so confused. It sounds like there are criteria for a suspicion of egpa or any other illness and if a patient is obviously seriously ill then tests need to be done to figure out what the problem is. I don't know why I can't get a diagnosis.
I am 60 years old
Asthma that got rapidly worse ten years ago
permanent lung damage and spots on lungs and bronchiestaces (spelling?)
Many surgeries for nasal polyps and ear infections , tubes etc
Hypereucinophilia
Brain damage from loss of blood flow
But my rhumatologist says she does not think I have egpa because my ANCA tests always are negative and egpa does not usually affect the brain. I don't really care what they decide to call it I just want to get appropriate treatment and feel better... sorry I'm ranting again
That me now my test come up negative but I sure do go in hospital for breathing oxygen levels low. Not a cold or anything. Back 2011 I had no immune check blood all levels bad thought it was lukemin but no then stayed a year in and out of hospital high fevers, swollen spleen, kidney hurt but no stones, then breathing, problems horsness, with poplys, now hole in nose but my rehmo, says I am healed from wg. Fatigue all the time, in and out if hospital still after having ritumb treatment for a month. I read that u can never heal just remission. I also read must have biosopy done to be sure, Dr never has. I am changing rehmo Dr cause mine thinks I am fine, but my body says no!
Lots of questions! From my 12 year experience with EGPA, here's my answers:
1. Any form of EGPA is vasculitis
2. Therefore, there is no form of EGPA that is not vasculitis. However, there is a 1999 study by Artes and Franco [and a couple of other studies] that suggest there is an EGPA-like syndrome that develops in asthma suffers who've been withdrawn from steroids like montelukast.
3. The eosinophils cause the formation of granules - called granulomas. It's the granules that clump together and have the potential to get big enough to break the small and medium blood vessels in major organs.
4. When nasal polyps are removed, they should be tested for the presence of eosinophils. I was told that this is standard practice in Canada, and that ENT doctors are trained to recognize elevated eosinophils. In my case, I had a polyectomy 6 months before experiencing heart and lung issues, which quickly led to my diagnoses [after 3 weeks in hospital].
5. Putting a tube into the lungs wouldn't be a usual diagnostic tool unless the doctor is certain that she/he can grab the eosinophilic infiltrates from eiosiophilic pneumonia. It is unclear to me how tissue would be obtained from what you're describing. My lung biopsy required the removal of tissue. It was inconclusive as I'd already been on prednisone for 2 weeks before the biopsy was done, so the tissue no longer showed eosinophils.
6. Most people who have active, uncontrolled EGPA are given intervention levels of prednisone in order to reduce the levels of eosinophils to appropriate levels [which should be 0 in full control]. However, I was started on 100 mg of prednisone and never reached 0. My best eosinophil level was .400 [top of normal] which was only achieved after two years of prednisone, supported by mycophenalate.
Hey thanks for the information. It sounds like you have been through a lot during your time with egpa. What a weird thing it is!!! I haven't given up hope yet although I have been pretty discouraged. I think that my rhumatologist may really help me. I sure hope so. I have been on fifty of prednizone for a week and then 25 for a week and then just back to three while I wait for the doctor to call me to tell me what to do next. I just started back on three today and I hope she calls tomorrow but who knows....doctors are spread pretty thin in Manitoba. Apparently my rhumatologist is going to get back to me after she goes over my file and I discovered that true to her word she actually did the paperwork already to get the file so that is sooooo good! I'm excited.....
Thanks for your gracious sharing. I'd like to have detailed information about the second answer you posted above. Since my boyfriend had been diagnosed with CSS by some doctors, and some other doctors thought his symptoms were not CSS related.
He developed asthma from 2012, then during 2013, he suddenly had stomach ache for no reason, vomited a lot,drastic weight loss, he had a lot health checks here in China. Only a few times with high eosinophil level, biopsy from suspicious red spot from skin showed nothing. With no organ damage, except for enteritis. Oh btw, he also took montelukast when he first developed asthma for treatment.
Doctor tried Methylprednisolone and Tacrolimus, his condition improved the day he took medicine. The doctor wasn't quite sure about the diagnosis in the first place, yet there seemed no other justified diagnosis for his condition. He, on the other hand, did try to seek advice from other doctors, their opinions were different.
Back in 2014, he almost stopped taking all medicine for his conditions were improved so well. Then he found out that his ex-wife cheated on him for several years, and the second son of his is not his blood. The most loving and caring man I've ever known went to the ICU the night he found out the news, he was only 31 years old.(It hurts me like hell to recall his past, I just can not imagine how painful and miserable he was before we met. ) Anyway, he had a full-blown relapse, no matter how many pills he took, what measures were used, his stomach ache couldn't be stopped until 9 months later. Till now, he takes Methylprednisolone (4mg), 1.5 tablets a day, plus Tacrolimus 1 capsule a day. His stomach has unwell feeling when sometimes he feels blue, yet most of the time he is doing okay, and he is cutting dose gradually.
Deep down in my heart, I wouldn't believe that he has CSS, his childhood and adult life have been not easy on him, he always tells me that he can't live a long life, I should leave him for a normal life, a healthy partner. He just won't believe that if he accepts scientific treatment, exercise regularly, with his condition, he can have a long life expectancy.
In China, we don't have much access to the most advanced technology, nor the newest medication. I've been searching for medical papers, yet find little. We don't have a single community for CSS people in China. I don't know where to find help and advice.
When I saw your second answer, I searched the paper, trying to find the evidence that he has no CSS, just EGPA-like symptoms, yet I still find not much. Although I have no idea what I could do even if he has no CSS, he still needs to take those medicines.
Sorry I've talked too much, I just feel so relieved that I found this place to share. He is love of my life and I will never give him up. I could be devastated sometimes feeling afraid that he will leave me any minute, please understand and forgive my mumbling.
I don't know much about the EGPA-like symptoms as I just recently heard about that alternative. I think doctors are understandably reluctant to diagnose CSS or EGPA since it is so rare. However, as you can see by this chat site that many people are confused, think they might have vasculitis but don't know which one, etc. The irony is that in order to save someone in such distress, the doctors give prednisone quickly. The problem with that is that the subsequent biopsies are usually inconclusive or show nothing -- since the eosinophils are then suppressed.
So there's no obvious answers to your questions. Stress does play an important part in most auto-immune disorders, so that needs to be factored in as well. Sorry I can't be of more help. I do find however that regular exercise helps. I play strenuous water volleyball 3 times a week for 2 hours each time. The water helps support my joints that have been damaged somewhat by the prednisone and the stretching and jumping helps reduce inflammation. I also eat a Mediterranean diet, which I believe helps [especially cutting out or reducing white bread, rice and sugar].
I encourage you to continue reading what you can and maybe something will make sense sooner rather than later.
Thanks SO MUCH for your prompt response and warm advice. I can't hold tears every time I think of his illness.
Yes I've been encouraging him to exercise regularly, and will do so. I'm so afraid that these pills he's been taking will hurt his organs. He deserves as everyone else a normal and happy life. He is only 36 years old, we haven't got married yet, I'm so scared.
Hi Cathy, It depends... My eosinophils were 2500 after the polypectomy, which is way too high. My ENT should have requested more testing. Instead, 6 months later, I had both lung and heart failure with eosinophils over 5000. I had extensive perinasal activity [including a middle ear infection and sinus infections] before I eventually came down with EGPA. So, I'd say that if nasal polyps contain significantly raised eosinophils, then it proves that there is a problem. Not necessarily EGPA but questions should be raised, especially if bronchitis or asthma is present.
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EGPA treatment
