I have a dilemma as I see 2 consultants from different hospitals- one is a rheumatologist from my local hospital and the other is a Vasculitis specialist from the rheumatology team at Manchester Royal. They both have very different opinions about whether I should come off my medication. I am on 75mg of aziathioprine and 10mg hydrocortisone. My aim has always been to get off most if my medication as I was diagnosed about 12 years ago with Churg Strauss or EGPA. My Manchester consultant agrees this is possible as I haven’t had a flare or infection for such a long time. My local rheumatologist advises against it and says I’m on a homeopathic dose and why take a gamble with the risk of having a flare and needing high doses of prednisolone again. Just wondered if you knew of any cases of people with my disease that had successfully come off medication?
I initially had cyclophosphamide and have been on high doses of steroids but reduced over time
Thank you
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Katie18
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Hi. My situation is very similar to yours, I’m still on 75 mg azathioprine and 5 mg prednisolone, after 14 years since diagnosis with EGPA (the new name for Churg Strauss). I have pushed to come off all medication so was sent to endocrinology to check if my adrenal glands are working, this is called a synacthen test. Sadly they don’t work so I have had to accept I’ll be on the steroids my whole life. I am gradually reducing the azathioprine, by 25 mg every 6 months, and hope to come off that completely one day. Good luck!
Thank you Chipper. My adrenal glands don’t work properly either so I’m on hydrocortisone. They have just done a test where they take blood from me every 2 hours for 12 hours to see if that’s still the case. One consultant I see is keen for me to reduce and come off aziathioprine if possible - the other one says it’s too much of a gamble. So I’m trying to find out if there is clinical evidence for either approach. Good luck with your care and thank you for replying to me
Hi Katie. I am on benralizumab and have managed to taper pred down to 2mg/day. I am hopeful of getting off pred but I suspect I will be on benra forever...who knows though. I am also on statins, antiplatelet and BP and suspect those won't be going away soon Also taking a ton of vit D . Before biologics I tried Myco and Aza but couldn't stomach either..especially the Aza. My best wishes for your journey. Lev
hi Katie18 I am on Mycophenolate 3000 mg per day. Also on benralizumab and betamethasone rather than pred which has never been effective for me. I am not convinced that the mycophenolate is effective as when I reduce the steroids I flare really quickly so have to increase ( I’m on equivalent of 25-35mg of pred). My rheumatologist thinks that the risk of coming off mycophenolate is too great. They did some basic bloods re cortisol which is low. I suspect adrenal function is low to non existent. I am trying a very slow taper on steroids ( over the next 6-12 months ) . The biggest issue I have is I have gained 10kg in fluid ( 46kg up to 56kg) and I enquired of my consultant pharmacist she agreed that gain is not caused by the steroids as betamethasone is used to decrease fluid ( which I’d does)
I also have rituxumab every 6 months …So to answer your question no not off all drugs and think it’s very unlikely to be honest.
I do have an appointment in the new vasculitis unit in London end of Feb with a consultant who was my respiratory specialist until 2 years ago so hope he has some smart ideas.
Good luck with everything - coping with the rare condition is very arduous and remember (as one of my nurses said) ask as many questions of your team as you need to be comfortable with the regime as you can !
Thank you - I will report back on here if there is much to add. Have you a local support group - we have 6 people in ours but no one with EGPA but it’s good to air ideas sometimes..
Yeah I was on myco for a good while and had the same huge dosage and like you did nothing for me except gave me a lot of pain. Never really was a viable steroid saving solution.
Hi EGPAGuy - quick question - what drug, if anything, were you given as an alternative to Mycophenolate? And if anything was its better as a steroid sparing treatment ? TIA - Elaine
Hi Elaine. After myco I was given azathioprine ramping up to 150mg/day. We also discussed Methotrexate but that never happened. Aza was awful for me but that's just me. You know that feeling when you have drunk too much and the room starts spinning. Aza at 150 was like that for me 24/7 without the fun of getting there 🙂. I came off it and back to 20 mg/day pred until I was finally given Mepo (tapered pred to 7.5) and then switched to Benra (still tapering pred and currently on 2). Best Lev
Excuse me but I have to disagree with your pharmacist: weight gain is a widely known side effect of prednisone! To the point that one develops so-called "moon face" weight gain in cheeks and fatty lump at the back of the neck top of the spine. I had read about this when I began taking 70mg dose Pred and sure enough it happened to me.A year later when I tapered off I was surprised to see that eventually little by the weight reduced and very slowly I've shed the 20 lb gain. The holiday pictures of my face alone from 2 years ago show the difference. I experience with Pred is not unique.
Hi churg01- correct prednisone is widely known to cause weight gain but betamethasone and dexamethasone are generally used to reduce oedema with certain patients. I have not been on Pred for a few years now as it did little to control my EGPA inflammation and (for me) betamethasone proved more effective. Obviously as patients we are all different…for instance since I have been on betamethasone I have had cataracts in both eyes which is something that didn’t happen with years on pred
I have EGPA and this time last year was doing really well having been on the MANDARA trial for Benralizumab, I was still on Methotrexate but down to 10mg a week and had been off prednisolone for 18 months . I always still had a few niggles but nothing too major so asked if I could come off methotrexate which was agreed with some caution. I felt great for a month or so and then all the old symptoms started coming back resulting in a full relapse and lots of pain and issues. The frustrating thing was I told the hospital I was having a flare and they kept dismissing it until I ended up back in A&E then they agreed with me and apologised .
Because I had relapsed I was taken off the Mandara trial even though I’d been on it over 3 years and now have Benralizumab once every 8 weeks instead of every 4 and still can’t understand why they think effectively halving the dose is an effective way forward
So it was back to 30mg of pred daily which calmed things but this time I asked to try an alternative to Methotrexate as it still wiped me out once a week so Mycophenolate was agreed .
As per ElaineRH’s reply I’m now on 3000mg of Myco daily and have been on the full dose for 11 weeks but as I’ve just tapered pred down again to 12.5mg a flare has started again so not sure it’s as effective as MTX for me
I’m still glad I tried coming off MTX though as you don’t know unless you try but you just have to be aware of your own body and watch for the signs and report any issues quickly.
Hi JGM1 I was already on Benra whilst the mandara trial was taking place although wasn’t on the trial. I asked if increasing the dose to every 4 weeks would give me remission and I was told quite bluntly that I will never find out as neither mepo or Benra will be licences for EGPA as nice deem that all patients will have access to both drugs via the severe asthma route and therefore another licence is unnecessary… helpful (not) ..
Yes exactly, that’s how I’m now receiving it via respiratory rather than Rheumatolgy so I’m basically getting the approved asthma treatment not EGPA. Interestingly I had a brief chat with an expert in the field who said a number of EGPA patients who had been withdrawn from Mandara are experiencing similar relapses after being placed on the 8 weekly treatment rather than 4 on the trial
agreed ! In addition my asthma hasn’t improved but they will still keep me on it as it’s the only drug that managed to suppressed my eosinophils (mepo didn’t work and I was very poorly and rislizumab caused anaphylaxis. )There are no plans to take me off it so that’s good
I think that’s partly why they didn’t believe I was relapsing as it does suppress eosinophils so it took a while for the blood results to reflect what I knew was happening already . ENT also don’t offer me any other treatment for worsening sinuses as they say Benra is what they believe to be a good treatment (doesn’t seem to matter if it isn’t actually working)
From what I have learnt even though the eosinophils can be suppressed once I entered the vascular phase as I appear to be, it seems like every one is fire fighting. My rheumatologist doesn’t just consider the bloods which to be fair aren’t stable anyway, and focuses on symptoms. I’d had many ent appointments over the years and I am only without sinus issues whilst on steroids. Maybe see if your team can add an ENT referral just to see if they can do anything to help.?
Thanks I do see ENT regularly but they just say it’s the EGPA causing the issues and defer to Rheumatology to treat . Interesting your Rheumatologist treats the symptoms not just the blood results as that’s exactly what I suggested should happen . To be fair it was Respiratory research at the time that ignored the symptoms until I was so bad they couldn’t .
Don’t get me wrong I’ve had some great care and treatment too just this last year has led to many frustrations and set backs
It’s often the same - my respiratory team were so focussed on my asthma phenotype that even though I said for 18months something else was going on especially with my heart I saw a cardiologist privately and she referred me on to the Brompton… only too 2 weeks for a diagnosis whilst admitted as lung function down to 22% and heart not too good. Since then the care has been generally good. Interesting to see what my old consultant says when I see him…
Interesting re benra. I was not on the trial but now on benra every 8 weeks and I have tapered very very slowly to 2mg/day. Taken me over a year from 5 to 2...
Glad it’s working for you , I thought it was for me but it’s obvious now it has to be balanced with other treatments to be effective in my case . I know from speaking to the research team I wasn’t alone in relapsing but also that Benralizumab was completely effective for others so hopefully you can remain in remission and getting off pred.
Hi, been on benralizumab for 8 months and 5 mg prd,taken a while for benra to settle everything down, Eosinophils still .00 after 8 weeks will start reducing Pred after sinus operation,. Been treated by immunologist and respiratory specialist have not had a good experience with rheumatologists but that's me. 👍
Hi Katie I share a similar story to you. Was diagnosed in 2016 after a rapid deterioration in health including adult onset asthma sinus problems . I spent 2 weeks in the renal unit of Derby City hospital. My kidneys were not the only organs to be involved, I also had brain and lung involvement ( which caused a pulmonary haemorrhage) but in terms of care, the renal specialists had more authority than the rheumatologist. I had intravenous steroids, plasma exchange and cyclophosphamide and steroids 60mg. You can only take the cyclophosphamide for 3 months, and I then went on to Azathioprine.
Having been so ill, I nevertheless achieved remission within 6 months. I do think the plasma exchange was one of the reasons for this.
Fast forward to now, and I am living in South Wales. I was transferred to the renal unit in Swansea, where I attend for my 3 monthly checks. Like you I have not had any relapses or flares, and the subject of medication reduction is one I constantly think about . I currently take 4mg prednisolone and 100mg Azathioprine daily. My renal consultant here has suggested I try reducing the azathioprine rather that the steroid, which is a low dose anyway, but as of one of your specialists has said why take the risk, and that is my concern, having been so ill in the first place. I notice you take 75mg azathioprine - have you always been on this dose-or have you reduced down from 100? I will follow the progress on this if you care to keep updating
Hello nanaC I have been reducing aziathioprine too by 25mg. The next step would be to reduce to 50mg. I have been really ill before so naturally I am concerned about reducing but I’m not sure how good it is for me being on this medication with its side effects. I was told from the start that I would get off medication and one of my consultants is still saying that’s a good plan - it’s a tough decision.
Hi. I was diagnosed with GPA about 16 years ago and ended up on steroids for 2 years and azathioprine for 11 years. I then came off medication completely, as the azathioprine was affecting my liver. I was fine for 2 years, until I got Covid. I believe that kicked it all off again and I'm now back on azathioprine, without any mention of coming off it again eventually. Could be your local consultant is right to keep you on a maintenance dose to avoid it all kicking off again.
Thank you for your thoughts. It is a worry coming off medication as because I was on aziathioprine during lockdown I was protected quite a bit by being given extra covid jabs etc. I’m sorry covid caused you to flare -I do believe getting infections is still a risk. I’d like to get more information about the number of people with EGPA who do come off medication & are ok - but it’s difficult to build a picture and everyone is so different - it’s a tough call to make.
This is a little to the side of above topic but to those of you having sinus issues caused by asthma/ high eosinophils: I can't recommend highly enough Nucala monthly injection. I understand that both Nucala and Fasenra (different name brands similar formula) are difficult to obtain and not approved in the UK. But try to get it when becomes available. Immediately after beginning this regiment my asthma/sinus symptoms disappeared supplemented with use of Trelegy brand inhaler. I tell everyone once you get that out of the way it's easier to concentrate on and tolerate the EPGA overload of medications, infusions, flare ups, nerve pain and whatever else you suffer from because at least you can breathe easier. I've had great success with this medication, I hope it becomes available to you in the UK soon.
Hi Churg. In the UK the only way to get Nucala (Mepo) or Fasenra (Benra) is from a diagnosis of eosinophilic asthma or severe asthma. Since most of us have one or both of those we do have access. The main problem is lack of knowledge of EGPA by hospitals and GPs. Most have never heard of the ailment or the biologics or its connection with adult onset asthma . Its down to luck to get a diagnosis early. I was initially told I had lung cancer by my local hospital! A PET scan put paid to that and the hospital just didn't believe what the radiologist said (vasculitis) and it was left to my wife and I to research and eventually identify a specialist and get my GP to write a referral. I got diagnosed really early because of it. I am grateful to now be under the care of a multi-discipline team who are either EGPA specialists or highly knowledgeable of vasculitis. It took me over a year to get biologics. He I would say the jury is out on whether rituximab or benra is the preferable treatment. Certainly hospitals were loath to give new patients rituximab during Covid but now of our small egpa group in uk (5) 3 are on benra, 1 on rituximab. We are all doing well. The 5th member we haven't heard from for a while. It's interesting to note though that one of us on Benra whilst reporting 0.0 eosinophils still had a big flare recently and has had to restart the long taper off pred again. Hope this is all useful.
I have EGPA and have been on Benra for over a year now. I have been off Pred for six months after a long taper. My eosinophils are 0.0. I recently had an eye examination that showed significant deterioration in the arteries in one eye, which has occurred since my EGPA “crisis” 18 months ago, and am waiting for an appointment with the eye clinic. So some indications of continuing Vasculitic problems. Waiting to hear if I need additional immune-suppressant meds. Despite all this, I’m generally feeling well.
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