I've just found this site., and this is my first post.
Do any of you have Churg Strauss Syndrome? Out of the blue in 2008 I developed sudden onset severe difficult asthma. It started with a severe, barking cough, 24/7 & my health has continued to deteriorate, with frequent emergency admissions,despite a NEBULIZER to use at home. I Was forced to accept I'll Health Retirement from my much loved job at the Met Office, and even Critical Illness paid out. I'm only 53.
In 2012 I suddenly developed severe muscle pain in my upper back, neck & shoulders. Morphine is the only medication which touches this pain,mwhich is constant.
In 2013, I saw a programme on Churg Strauss Syndrome on Mystery Diseases on Sky Discovery Channel. it was truly a "lightbulb" moment. So many similarities to me, it was unbelievable.
My GP of 37 years says he had never seen it, but vaguely remembers hearing about its discovery when in Medical School in 60s.
My new Respiratory consultant here in Jersey, unbelieveably turns out to be an authority on the disease& agrees with my concerns. Altho there is no definitive test, I have 3 3/4 mof the required 4'specific symptoms for a diagnosis. Altho a biopsy is impossible, as, due to my constant steroid dose, together with max dose of Mycofenalate, this will render it negative, my Consultant is treating me for Churg.
Anyone here with this diagnosis too??
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I've had CSS for past 27 mths and have researched a lot it about during that time. I also refer those with a potential dx to check their 'symptoms' against the Six Criteria used to assist dx of this rare type of vasculitis.
This link from the Mayo Clinic, a highly rated specialist US hospital, shows the six criteria used for dx. If you have at least 4 of these 6 criteria, then it is highly like;y you have CSS.
It turns out I have 1,2,4 & 5 of your list. I have a shadow on my lower R lung, but the Consultant said it would be like trying to chase a ghost to get a biopsy, and, as with the Nasal sinus biopsy (CT shows them almost entirely blocked., I've never been able to breathe thru my nose, since a kid, but it's never been looked into..) as I am on steroids & MMF, which are suppressing my immune system to control the eucinophils, any biopsy is likely to be negative. Southampton Hosp, said they would have to stop treatment, let me deteriorate, take a biopsy, then try to get it under control again.. No thanks!! Also, due to the severe Asthma, I'm unable to have anaesthetic..
I'm interested in finding out as much as possible about Churg, but don't want to become obsessed with it. I'm confident in my new Consultant, and am able to contact him at any time with concerns or any symptoms I notice, and he gets back to me the same day..
Under no circumstances let them stop the treatment. The inflammation within your body will continue to cause damage without prednisolone or whatever you are prescribed. I had the same symptoms as you and my hospital did a bronchospy and took lung biospy that way. Are you allergic to anaesthetic then? Only I have chronic asthma and have local and generals all the time.
Being ANCA negative doesn't mean you are safe from Churg Strauss Syndrome. It is simply one of many tests to get a clearer diagnosis. ANCA negative patients are prone to heart complications, I know, after 10 years of living with CSS I was diagnosed with severe cardiomyopathy and severe heart failure this year.
You need to be obssessed by vasculitis because it is sinister in nature and can damage even when it is in remission. Learn as much as you can because doctors know very little about the disease. I think the members on this forum know more than most doctors because we live and breath it!
I have been seeing a rheumatologist consultant for the past three years and after a seven week admission to hospital last year I was diagnosed with CSS. After seeing the same consultant in January he then said he was unsure of that diagnosis because I didn't have all the symptoms. He is now treating me for vasculitis, I am on a reducing dose of prednisolone I started on 60mg last September and I am now on
10 mg and see the consultant in September. I also take azaiathoprine 50mg twice a day. I was diagnosed with asthma 15 years ago, I have frequent bout's of sinusitis and I am waiting for an appointment to see an ENT specialist my doctor thinks I need to have my sinuses washed out, also been diagnosed with peripheral neuropothy and I can't feel the bottom of my feet and have very little sensation in my hands but I have what I describe as tingling in my hands and feet but if I touch anything it is like electric shocks in my fingers, also have patches of numbness on my arms and legs. I have a buzzing feeling in my stomach which is very strange and my doctor doesn't know what to make of it when i tell him. I do not know what my blood markers are but I will ask him he next time i see him. I have been treated with pneumonia only three months ago. So I don't know what to make of this whether I have Churgs or not as the consultant says to get a proper diagnosis I would have to have a brain biopsy which I don't like the sound of.
I don't know if this helps or not as I would like a diagnosis of what type of vasculitis I have.
You and I have many similarities and it sounds like Churg to me. Altho I Dont have the neuropathy, I was tested for it last year. I have never been able to breathe thru my nose, since a kid, and thought it was Adanoids. The consultant said a nasal problem was a primary symptom for Churg, together with severe Asthma, and on doing a CT it was found my sinuses are almost entirely blocked, which is significant. He said there was little point in doing a Sinus washout as the benefits would only be a couple of months, and it's a nasty operation.
I am on 10mg of Pred, having been on 40mg for years., for severe brittle asthma that they couldn't control (with another respiratory consultant who has thankfully retired) and have been told I must NEVER go below this dose. This is together with the 1g Mycofenalate Mofetil.
I was told that a positive nasal biopsy would be the clear decider for a Churg diagnosis, but it is neither here nor there, as I have other symptoms which are too significant to ignore, and a getting a biopsy from a shadow on my lung too, would be like "chasing a ghost"
My new Consultant, who specialises in Churg & Vasculitis said it would be dangerous NOT to treat me for Churg just because they haven't got this biopsy. Of the symptoms I have 1) severe difficult Asthma, 2) Very Hi Eucinophils if not suppressed by Steroids & MMF 3) Nasal sinus permanent blockage.. & I can't remember the other!!
I have very rigid fortnightly blood test to check the eucinophil levels are still under control, together with liver and kidney function levels etc as the Mycofenalate is highly toxic, but it is the only medication that has been able to control my "difficult" asthma.
I certainly would NOT allow them to try for a Brain Biopsy! If you are on Steroids it's going to be negative anyway. Your consultant should be prepared to treat you for it with the symptoms you have..
Churg covers a whole host of symptoms, that not everyone has. I have the severe muscular pain.. Last year, when my breathing was so poor, he was testing me for Heart Failure, in case the Churg had affected my heart. Thankfully not, but a very scary time.
Altho still have severe breathlessness on the slightest exertion, which he's now putting down to "deconditioning" as I'm not Asthmatic at the mo, but have got pretty frail in the last 5 years, I am "delicately stable", which is GOOD!
My new Consultant is from UAE & ijust happens to be an authority on Churg.. And a lovely man! .. He told me that 2 years ago he gave a presentation to 1000 Drs in Canada on Churg & they are trying to raise awareness of the condition. Maybe your consultant could speak to him..
How are you health wise on a day to day basis? What do you take for the pain? I have numerous side effects from long term use of Steroids, inc sudden severe Catarracts, & steroid induced Diabetes. Of course, I'm as big as a house now too, but if it wasn't for the steroids, I wouldn't be here now. A small price to pay..
I've never had Asthma in my life, nor smoked. It all started, very suddenly, with a barking cough, with dramatic deterioration..
Bestest wishes, Ann. This is all so hard to cope with.. It's taken over my life..
I have 4 out of the 6 stated and a good amount of the symptoms but my consultant says that he is unsure it is CSS and is treating me for Vasculitis. Whenever I say that things are not getting any better or my hands in particular getting worse he blaims the fact I have been steroids for some time and I need to reduce them I was on 30mg last year and I am now on 10mg.
As I have just changed my own Dr after moving house I am at a standstill for now.
I think you need to find someone who is familiar with vasculitis and in particular with CSS. From what you have said it sounds like they do not know what they are talking about.
However, you said ..."my consultant says that he is unsure it is CSS and is treating me for Vasculitis"
-> so in what way is the treatment different ... I just don't understand? CSS 'is' vasculitis and is just one particular variant or type of it. Both are treated using Prednisolone at a varying level to reduce the inflammation, plus an immuno-suppressant to calm the immune system down.
Re ..."Whenever I say that things are not getting any better or my hands in particular getting worse he blames the fact I have been on steroids for some time and I need to reduce them".
-> I would say that is totally wrong, and it is likely due to the steroid dose being "too low", and so need to increase, not "reduce them".
I doesn't sound to me that your current treatment regime is controlling your Vasculitis well if your peripheral neuropathy is worsening. Did you ever have Cyclophosamide to induce remission?
I wonder how much experience your Rheumatologist has of Vasculitis as it is very rare, CSS in particular. Have you ever had an opinion from a multi disciplinary Vasculitis clinic?
No I never had cyclophosamide although it was discussed at the beginning of the treatment and it was decided to start azaiathoprine. After seeing my consultant earlier in the year when he was undecided it was CSS whenever I told him about my hands and feet that were no better and my hands worse he said it was because of the steroids and I needed to reduce them, at that point I was on 30mg of prednisolone daily.
I have never had another opinion but my previous Dr was looking into finding a Dr with more specialist knowledge. Unfortunately I have moved house since and have changed Drs 2months ago so still new to the surgery.
You are entitled to a second opinion. It's difficult questioning a Dr's knowledge but worth it to get answers and more effective treatment. Please think about getting in touch.
Don't worry about a brain biopsy - I've just had one for what we thought was a probable tumour, CNS vasculitis, something else inflamatory and a multitude of other possibilities including Churgs. It's usually the last resort though!
YOU make an informed decision .... the rest of us can only have anecdotal opinions as we're not Doctors !
It wasn't half as bad as I thought it was going to be - the FEAR was worse than the procedure although I was warned of the main risks - stroke, seizures and a brain bleed - I came out unscathed although had to remain on triple AEDs for ages but then that's my 'case'and you may be entirely different.
It didn't however give me a definitive diagnosis and sadly I still remain a mystery ? "Something rare with abnormal multiple cells multiplying very slowly so needing vigilant monitoring with MRI s "
Righty oh ! That's that then ! Who knows where it will go.....and when ???
I've lots of other areas of abnormality but they can't biopsy them all nor would I agree to it !!!
I take it an MRI has proved negative for you ?
If you decide to go ahead (it's an entirely elective diagnostic surgery) be prepared for the fact that you still might not get a definitive diagnosis.
These rare diseases are very elusive and I find it difficult coping with the uncertainty - I'd love a title and therefore a treatment plan ......with certainty and therefore a prognosis . It's not to be ........at the moment anyway.
However I'm a tenacious detective and am prepared to do my own research. Exhausting though !!!
Sometimes the Medics just don't know (and when it's a rare disease that's all they can know) so rather than just do nothing they hedge their bets and treat with steroids and immunosuppressants - as seems to be the case with you.
I'm still trying to understand the whole autoimmune thing so I'm sure there's another member here can give you a better opinion than me?
Ive a strong family history of autoimmune stuff not that Docs take a blind bit of notice of that ....I'm convinced there's something else inflammatory going on !!!
As I said don't overthink the brain biopsy nor worry too much - once you've decided it's an option for a possible diagnosis then get the relevant Stats re Churgs (more detective work) and decide if it's worth your while ?
I'm so sorry you've had to stop work etc etc It's dealing with a reduced quality of life makes for mind altering big effort.
I can take you through the biopsy thing if you decide to go for it - just pm me.
Take care
Susanmac
Oh I should mention I've also got steroid resistant brittle Asthma and the only thing helped was a 6 month course of strong antibiotics - so my lungs are behaving themselves at the moment - first time in over 40 years !
Respiratory Consultant says my lungs breed nasty bugs and of course bronchiectasis has now developed probably due to multiple infections and long term use of inhaled and occasional systemic use of steroids !!
Honestly I don't think there's a bit of me that works properly yet I'm slim fit and relatively active ! Can't win but we have to keep trying.
If you happen to read my previous posts they're kind of out the window now and I'm back to the big bubble of no answer ! Take care Ann
As I said earlier, I've found out a great deal about CSS over the past 2+ yrs, and would highly recommend one particular 'closed' Facebook group called "Churg Strauss Friends". It was started by an lady in the US a few years ago and now has 575 members, with many coming from UK and Australia.
Some of the members are real experts having had CSS for as many as 25 yrs in some cases. I have found out a tremendous about this disease from their postings, since they nearly all have slightly different symptoms & experience to share.
If you have any further questions, please ask & I can dig into my knowledge bank to answer if possible.
BTW: I'm currently on 9 mg Pred & 150 mg Azathioprine, and few symptoms (numb small toes on both feet & breathlessness on exertion). But luckily I appear to have a 'mild' severity (started on 30 mg pred) and no organ damage other than some neuropathy & lung deterioration as mentioned.
Keep well & keep fighting .. a positive spirit definitely helps.....
I'm actually in hospital being investigated for Churg Struss Syndrome.
In fact tomorrow, ops it is 4.59am I'm having my first chimo treatment for it.
I'm a 58 year old lady. I've hadn't asthma from the age of 13. Sinusitis for the past 10 years. Urticaria 2 bouts over 2 years. now my right leg is so painful, difficult to walk. I've had nerve conduction test that show mononucleosis multiplex, this has been very aggressive so no messing chimo even before byopsy.
I'll keep you posted on how I get on. Unfortunately my sister died of luckimia and obviously this is one of the many side effects of the chimo, but there is no other option.
Will let you know how my 1st bout goes.
A very scared lady, wish my dog could be with me. Lol
Good luck with your first chimo. I'm sorry you are having such a rough time. Hopefully you will soon be feeling much better. I'm lucky that the Churg seems to be controlled by the Pred & Mycofenalate dose which was started pretty promptly, and tested negative for the neuropathy tests.
I will be thinking of you today. I know exactly how you feel about your Dog.
I live alone, and my 4 cats are my life, and they really help me cope..,Such characters.. (No, I'm not allergic to them, thank goodness!)
Brilliant article , it tells it how how it is . i am here in Australia and have been treated for CSS for 2 and 1/2 years , it took the top medico in Aust 3 years to work out what I have . Although I have about 80% of the CSS complaints my Chest , feet sinuses kidneys etc and bad response to movement and balance , another section of our leading hospital believes I am being wrongly treated but the basic issue is to long in a very stressful position . I find morphine when needed or endone and patches as helpful but relief is virtually impossible . The Asthma and Flu/pnumonia I end up with every time someone near me sneezes . and i am out of action for weeks with endless courses of antibiotics and 50mg of prednizone for 3 or 4 days , I am fed up doctors have no idea of the battles each day brings and working under pain in order to survive is the underbelly of the disease its just more pressure and then keeping a roof over your head is madness as you bleed to death financially .
I have not seen the Doco on Churg strauss on medical mysteries and would be keen to see it . Can you supply a link so i can view it . i am taking a copy of your post and putting it in front of my immune specialist 40 times in hospital and mostly in isolation as the disease is acute and our immune systems have been turned off so we be near other very sick people worse and like wise we cannot be in a situation to be infected by them , so Hospitals are not for us maybe a desert island .
Life can be such a rotter at least i made 55 I hate seeing children sick.
I've put in some suggestions to help with those chest bugs and pain.
My own DX and treatment.
My Churg was diagnosed in 2007 following firstly, increasing asthma, then really severe rhinitis, then what felt like the Worst Flu Ever, then bleeding around eyes, bleeding from bowel, inflammation of pericardium, nerve damage (mononeuritis multiplex (feet, balance, hands) as with all who have commented here), progressive lung damage. My Definitive DX was via a nasal biopsy which found the required extravasated eosinophils -as the other 5 signs were all there. Treated with Cyclophosphamide to smack the Churg down and then on to maintenance with preds and other meds that didn't work / made me more ill. So prednisolone it is for now! (I'm osteopenic and have a bit of cataracts too)
LOTS OF CHEST BUGS (initially) AND HOW I FIXED THIS
Progressive lung probs initially meant I got a chest 'bug' roughly every month, had to take lots of antibiotics and I saw that my lung functions were declining a bit more every time they were tested. I was worried I can tell you, saw my wooden box looming in the near future!
so I take VITAMIN D FOR CHEST BUGS and I'm getting back some LUNG FUNCTION but see below re flu jab.
I have managed to turn the chest bug problem around by taking 5,000 i.u. vitamin D a day (please read up the research reports on how it works) so I now only get bronchitis about once a year, AND I think my lungs have healed quite a bit as the lung plethysmography has shown an improvement from the nadir of only having 30% of predicted FEV1 etc a couple of years ago, to around 50% 6 months ago. Belive you me, this makes a big difference to what you can do. I also got back some nerve function and reflexes which was not expected by the neuro specialist so quite pleased about that! Not fully working yet but I remain hopeful especially re the pain.
FLU JAB SIDE EFFECTS
All had been improving good-ish ...up till a few weeks ago, when I had the annual flu jab and noticed that the lungs immediately felt a lot tighter and the usual ventolin wouldn't touch it. I had my plethysmo tests again just today and the lung function has dipped 10% by most of the measures, however it doesn't feel like it's getting any worse now. Just have to try not to talk at the same time as walking!
TAPERING PREDS etc
Currently tapering down the prednisolone and I am on 11 mg a day. Target 7.5 mg / day. Hope that will help the bones more than the calcium tabs do. I have had a course of Rituximab but it hasn't touched the eosinophils - they're pretty much back on song (i.e. a bit above normal) a month after the treatment so I don't think this is for me. Blood tests regularly to keep an eye on the blighters.
Methotrexate made me ill, immediate effect on lungs and muscle strength. Mycophenolate and Azathioprine ditto. I'm ANCA negative.
LYRICA (pregabalin) FOR PAIN
I take Lyrica 100 mg a.m. and 150 mg p.m. for the unending pain in the feet from the nerve damage and it makes it much more bearable, so I'd recommend it. I take the smaller dose in the morning otherwise I'm too sleepy around mid-day. I take Flixotide (500 mcg dry powder steroid) twice a day for asthma, to reduce lung inflammation.
VENTOLIN = PROBLEMS?
I take Ventolin to open up the airways. Can't take long acting any of the recommended long-acting bronchodilator meds (LABA's) as we found these trigger bronchiolitis / alveolitis after a couple of days use, no matter if they're dry powder or pressurised type.
HOWEVER I now THINK the ventolin may do the same, paradoxically - the other day I noticed that if I deliberately sniffed a fraction of a puff of ventolin, trying to pin down why I am so 'sinusey' an a daily cycle -my nose became blocked almost immediately, with lots of snot with white cells soon after. ...and considering the airway is considered to be all one (nasal, sinus, lungs) as far as allergies go I might be on to something here. I even wonder: What if ventolin can cause Churg, it being considered by many to be an extreme form of allergic condition? Anyway since then, I've been holding my nose when using the inhaler to minimise the chance of getting ventolin in there when exhaling after a 'puff' and INCREDIBLY my sinusey-ness has almost gone and my sense of smell is back. Just in the last couple of days since starting to do this. Wonder if it's making my lungs bad... Wonder if there's a diff bronchodilator that won't do the same...
SENSE OF SMELL
Normally I have to be on 25 mg preds or more to get my sense of smell back, but keeping the bronchodilator out of my nose has brought it back even on the low current dose of preds. Whoo-hoo! Has anyone else noticed this effect from bronchodilators?
FLU JAB thoughts...
I also wondered if the 'flu' jab made things, especially my breathing and nerves, worse -as it contains a universal adjuvant to 'ramp up' the immune response, and maybe that's the last thing we need as it's an immune system that's primed to attack ourselves already? Discussed not taking flu jab next year with GP as the side effects have been so bad this year, but he says, 'on balance, take the jab, better than pneumonia complication / hospital etc.'. I'm not so sure. He said they do have some antivirals if you still get the flu anyway.
NEUTROPHILS
I always have had a high neutrophil count - g'teed every blood test - and wonder if these are recruited in response to an allergen e.g. maybe it's the Ventolin? Am going to discuss with the consultant when I can get an appointment. But is there an alternative to Ventolin / salbutomol if this is what's ramping them up?... been taking it for years since developing wood-dust asthma as a teacher.
And now: ACROMEGALY. Nothing to do with Churg. Do wish things would improve / leave me alone - just recently DX with too much IGF1 from a pituitary tumour, found incidentally during investigations for loss of hearing. Da#n it. Any readers had one of these treated? Maybe it's been triggered by the azathioprine, who knows?
So, things can get better, and other things can come along to annoy you!
Any ideas / thoughts, dear fellow vasculitis sufferers?
Update from me re the ventolin link: I've seen the asthma nurse and the chest specialist as the breathing levels were getting silly: from being someone with a regular peak flow of 700 l/min I got down to 350 l/min and believe me, that makes it hard to do anything exertive. Changed to theophylline pills + aclidinium bromide (antimuscarinic) inhaler, + Bricanyl (terbutaline) inhaler, + fluticasone inhaler. All dry powder inhalers. This has made a MEGA difference, I hit a peak flow of 450 a couple of days ago and feel I can start to get around more. Hope this helps anyone struggling with their Churg Strauss asthma (the two go hand in hand!)
I was on most of these inhalers & the Theiphilline before Churg was even considered. (I saw it on Mystery Diseases on Sky & like a Lightbulb moment, & got the doc to do tests..) They like to call mine " difficult Asthma" but has been MASSIVELY improved by MYCOFENALATE MOFETIL so I no longer need to carry a portable Nebulizer wherever I go, but this drug is highly TOXIC, & altho I've only been on it for just coming up to 2 years, I'm becoming super sensitive to the side effects in the last few months. The docs don't think I will be able to continue on it for long. Scary alternative just relying on steroids & plethora meds & inhalers that have become increasingly useless. Apparently a new drug in inj form has recently finished trials & should be released late next Summer. Holding out for that, & that it works, otherwise it's back to frequent Emergency Admissions with Asthma attacks, with peak flow as low as 90. My best, & now stable @250 approx. V poor quality of life even with this,Jones fractures in both feet due to steroids, I'll Health Retired, isolated, feel about 90 but only 53. Also DNR. What's the point, in prolonging the agony?? That's how I feel.. Grim! Stay well!
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