Churg-Strauss Syndrome – Warning Signs

The rare but potentially fatal condition of Allergic Granulomatosis, also named Churg-Strauss Syndrome after the two scientists who discovered it, is generally not recognised or diagnosed until a life-threatening bleed has occurred inside a major organ of the body.

The primary method of diagnosing Churg-Strauss is not however from the deadly bleed itself, but rather through the results of a particular blood test called ANCA (anti-neutrophilic cytoplasmic antibodies). It takes about a week for the results to come back from this test and most surgeries and many hospitals will have to send the blood away for this analysis. Because of the duration that this test takes it is particularly important to have the test done as soon as there is reasonable cause for concern.

For Ladan, my wife, the results arrived after she had started bleeding in her head and less than an hour before she fell unconscious. Most of what I am about to write is based on what were, with retrospective vision, probably symptoms of Churg-Strauss, in the hope that if you have found this article by searching for symptoms in a search engine, and the picture seems to fit your case, you might ask for an ANCA test to be taken as a precaution. These symptoms are summarised toward the end of this article.

It is important to note that I am not a doctor and the symptoms listed in this article are not necessarily recognised by the medical community as being related to Churg-Strauss Syndrome, however given the devastation that this rare condition can cause I believe that existence of two or more of these symptoms together would make it prudent to check for Churg-Strauss or a similar disease of the auto-immune system. Most web sites dealing with Churg-Strauss Syndrome do not suggest what the early symptoms may be beyond asthma, I can see, retrospectively, that there were many signs of a worsening vasculitis and greater awareness of such signs may help to save others from developing the syndrome to the point that it threatens their lives.

Incidence of Churg-Strauss Syndrome is thought to be less than 1 in 100,000. It is classed as an auto-immune disorder as it flares when the body’s auto-immune system becomes self-destructive. It is a rare form of vasculitis that causes swelling in the smaller blood vessels of the body, generally focussing itself in a very localised fashion. It may affect other parts of the body before inflicting itself on part of a major organ which can then result in an internal bleed which, in turn, may result in death.

It seems that nobody is entirely sure what causes Churg-Strauss but a favourite theory is that a dependence on steroids may be partly to blame, and a group of people who seem particularly at risk from the disease are asthma sufferers, who tend to take inhalers containing steroids on a frequent basis.

Some studies have shown that asthma sufferers have often developed Churg-Strauss shortly after changing their inhalers, this raised questions over whether or not the new inhalers were causing the disease but further research suggested it was more likely to be the cessation of using the previous steroidal inhaler that was bringing Churg-Strauss to the fore.

Inhaler changes tend to be made following bad asthma attacks, therefore bad asthma attacks are often part of the recent history of a Churg-Strauss sufferer. It is also however widely thought that Churg-Strauss can cause asthma or asthma-like symptoms, so an apparent worsening of asthma may well be caused by Churg-Strauss itself, and if, in those rare cases, the steroidal inhalers are not working well then the change to a non-steroidal inhaler is likely to make the Churg-Strauss much worse. Perhaps paradoxically, Ladan found that her new non-steroidal inhaler worked wonders for her asthma, which perhaps suggests that it is the asthma that was worse rather than Churg-Strauss causing it.

Following a few severe asthma attacks which led to an inhaler change, Ladan’s first physicsl symptoms of something not being quite right were sharp pains in her arms and legs, these were fairly short lasting but occurred several times a week.

Next came the swelling. At first it was thought that an unusual insect had got a bite at Ladan’s right foot and ankle as they swelled up slightly, not like a balloon, but it was red and painful and several blisters formed in the effected area. Shortly after this the same pattern started to occur, though to a slightly lesser extent, on Ladan’s left foot and ankle. A cortico-steroid cream was used which helped alleviate the pain and a course of oral steroids eventually made the problem go away very quickly.

Another symptom that started developing around this time was a tingling and numbness in the fingers which sometimes also extended to the fore-arms, again slightly more prominent on one side but often reflected to a slightly lesser extent on the other side. As with the sharp pains this symptom started occurring regularly but it could last for quite a prolonged period of time. This was never really diagnosed as anything with a non external cause.

Ladan also found that her sense of smell went, whether this was a permanent thing that she becamew aware of occasionally or an occasional symptom I am not sure.

Swelling then also occurred in Ladan’s wrists and hands, again red and painful with several blisters forming in the affected areas, and again more prominent on one side but reflected to a slightly lesser extent on the other. In both the swelling on the wrists and on the feet the skin was raised by about 1 to 3mm with the redness. The combination of swelling and tingling aroused suspicions of an allergy, perhaps to a soap or washing powder that was in use, but again an oral course of steroids brought the swelling down.

Blood tests showed that Ladan had a very high eosinophil count (an inflammatory marker in the blood) while the swelling was active which came down quickly with steroid use. This is typical of Churg-Strauss but can also be caused by many other conditions.

The above symptoms were bad enough to be causing serious distress to Ladan, for example the swelling in her feet and ankles made driving and too much walking very uncomfortable. But the most distressing symptom was an agonising stomach pain that hit her about 5 weeks before the condition reached its life-threatening peak.

The stomach pain was in the top centre of her stomach, just below her ribs. It would start hurting about 1-2 hours after eating any food and would only be alleviated after a lot of vomiting. Barely any food stayed down during this period. Ladan had previously suffered from irritable bowel syndrome, another condition for which she had taken steroids from time to time, and knew that this pain was something quite different. It kept her bed ridden and was misdiagnosed consistently as gastroenteritis, though the possibility of a stomach ulcer or appendicitis were also considered.

In addition to typical treatments for gastric problems, an oral course of steroids was attempted at one point during this period but it is possible that many of the tablets did not stay down long enough to have an effect.

The tingling sensation in the fingers started to become more prominent to the extent that for a lot of the time the two fingers nearest the thumb on each hand, but particularly the left hand, felt almost completely numb.

A similar irritation occurred in Ladan’s face the night before a morning where she suddenly had a very severe headache with slurring of speech and blurring of vision, while a migraine can apparently cause these symptoms the actual cause was a stroke, the beginning of a brain haemorrhage that was to make Ladan’s condition critical before the doctors were even certain that she had anything more than an upset stomach. These last symptoms, of headache and effected vision and speech, are typical of a stroke and in any case (not just where Churg-Strauss is suspected) where you are experiencing these symptoms – and do not suffer them regularly as part of a diagnosed migraine problem – they should be reported to a doctor immediately as an emergency. Even doctors themselves are good at misdiagnosing strokes as nothing more than a bad headache, they initially made that mistake with Ladan and I have heard of other cases where people have walked into their GPs, following advise of posters on the wall at their local doctor’s practise, and gone home having been assured that they had a bad headache only to fall prey to what was actually a stroke later in the day. If you have such symptoms and they do not feel right then ask your doctor to rule out the possibility of a stroke as best they can, this should involve some strange tests such as having something scraped on the soles of your feet and being asked to hold your arms up and keep them level in the same position with your eyes closed.

Finally, one symptom that Ladan had before all those I have mentioned already, which persisted to the last, was frequent and vivid visions of something terrible happening to her. This may not sound like a scientific symptom but since Churg-Strauss could be causing very localised symptoms in the brain it may have had a physical cause, but in any case when you have clear visions of impending calamity and you cannot identify a good reason for them I don’t think it should be ignored. Ladan didn’t think so either so she talked to a GP about it, unfortunately it was just thought to be related to stress.

I hope I have painted a sufficient picture of what Ladan went through to assist anybody with similar symptoms in deciding whether to ask for an ANCA test.

Unofficial potential symptoms of Churg-Strauss Syndrome
Initial localised symptoms of a vasculitis will vary greatly between sufferers, I would recommend asking for an ANCA test when two or more of the following symptoms are present:

  • Recent alteration of a long-term regime of steroids, eg. changing from an old asthma inhaler to a newer one.
  • External redness and moderate swelling, possibly accompanied by blisters, localised to one location or mirrored to the same location on the opposing side (left/right) of the body
  • Tingling or numbness in the fingers and/or fore-arms. (Not from external pressure such as clothing, furniture or resting fore-arms on bars). This is also called neuropathy.
  • Tingling or numbness in the face.
  • Severe stomach pain that is not alleviated by treatments for sickness, ulcers or gastroenteritis.
  • Frequent vomiting that is not alleviated by anti-sickness medications.
  • Troubling visions
  • Temporary relief of symptoms by steroids
  • Stroke symptoms (severe headache and/or blurred vision and/or slurred speech)
  • Frequent sharp pains apparently coming from organs or deep inside limbs.
  • Loss of sense of smell.

With exception of the first symptom, most of these warrant a visit to the doctor in themselves, if two or more are present and particularly if there is asthma or a history of frequent steroid use which has changed within the last year then it would be worth having a doctor carry out an ANCA test. ANCA results vary according to how active the Churg-Strauss is at the time of the test and the results will not look as bad following a course of steroids when the syndrome is causing external swelling as the ANCA results will look just before a life-threatening bleed will take place, but the results should still show some evidence of the syndrome if it is present and a suitable immuno-suppressant treatment and/or monitoring can be started to ensure that the condition does not worsen. Once diagnosed the syndrome is not very difficult to keep under control.

This blog entry allows for comments, if you feel you can add additional useful information to this article, for example to help people recognise a potential case of Churg-Strauss syndrome, please do comment.

Link: About.com – Churg Strauss Syndrome
Link: BBC Health – Churg-Strauss Syndrome
Link: Churg-Strauss Syndrome International Support Group

14 thoughts on “Churg-Strauss Syndrome – Warning Signs”

  1. James, I stumbled on you site after googling Churg Struass as my specialist thinks I have it. This account is so detailed and helpful I have printed it out to read over again. I have bad asthma and developed Esinophillia after changing to Seretide inhaler and being given big doses of Steroids in January this year.
    I shed tears over your moving account of your wife, you are obviously a wonderful caring person and m heart goes out to you both.
    Thank you for helping me and raising awareness of this dangerous condition.

  2. hello james,
    i have had this condition for the past 10 years. mine started as flu like symptoms then painful tingling in my legs, which became weak and i developed ulna nerve palsy ( the nerve to my ring and little finger on my right hand was damaged) my fingers contracted and i couldn’t move them and my arm became so weak i couldn’t move it without using my other hand to do so. i spent a week in bed, all i could manage was fluids my apetite disappeared and i was constantly sick. i la=ost 21/2 stone in a matter of weeks. it was very frightening because my doctor thought i had a virus but when it hadn’t improved after a week i was refered to a specialist who months later diagnosed me

  3. Hi James,
    thanks for putting this up. My dad’s just been diagnosed with Churg-Strauss Syndrom (CSS) after suffering a variety of symptons…and I’m looking everywhere for information.

    The tipping point for him
    was a numbness in his feet. So, along with the forearms and fingers mentioned above, I think any extemity can be hit.
    As I understand: the condition is the immune system going wrong and attacking normal cells.
    Obviously, numbness anywhere should be treated urgently.

    He has a history of asthma and had an operation to remove nasal polyps.

    I think anyone at this point should ask for a blood check to look for CSS. As you say, the name of the game is early identification. The numbness in his feet lead to urgent hospitalization.

    As far as I can tell docs consider CSS rare. But it seems to be hard to diagnose and so warning signs are missed, or docs wait for all three ‘classic’ symptons to appear: history of asthma, skin rash and nerve damage(numbness)

    He has ‘drop-foot’ at the moment..can’t physically raise his foot from the ankle..making walking difficult. His neuro-surgeon said it would have been likely that he would have lost the use of his feet and hands if the condition hadn’t been treated.

    Now he is to be treated with Prednisone..Plz check my spelling (different name in UK)…a strong steroid which has been highly effective in dealing with the symptons.

    I think the problem may be that the symptons may be addressed..but the original reason for the immune system attacking wrong cells isn’t addressed.

    There is good account on wwww.abbottsys.com/churg-strauss.html

    He mentions diet. Antioxidants

    thanks again
    Ivan
    Another sympton is the skin rash. This is mentioned in other articles as a sympton of CSS

  4. CSS sufferer for the past twelve years. Didn’t ever notice the symptoms; had a stroke and was paralysed. Then after prolonged steroid therapy, recovered, to be struck down again – lost speech and use of the right hand. Now sufficiently recovered from all that, holding a full time job, functioning OK 🙂
    But still have aches, pains, rashes, asthma, cataract……..you name it, I have it!

  5. Hi James. I just wanted to thank you for writing this – we have been struggling with a diagnosis for my husband for about 1 1/2 months now – the strange thing is, he has no history of asthma and he is only 31. They now “think” that he has Churg-Strauss. He is on 100 mg Prednisone and has been on it almost the whole time he has been sick. We are so worried! Thanks for the info and if anyone has any other info, I would greatly appreciate it – please email me at bandycaine@hotmail.com
    Thanks again!!

  6. I was disgnosed with CSS in February 2004, after being hospitalized with what was initially thought to be a heart attack or pneumonia – I had gone to emergency room with chest pains and extreme shortne4ss of breath. Heart tests showed no blockage, so Doctors turned to blood work, where they discovered extremely high levels of esinophils. I was lucky to have the teams of doctors at Walter Reed in Washington to treat me–had I not, my condition might not have been diagnosed. The CSS had attacked my heart and lungs, and gave same symptoms as a heart attack. I have had asthma for 20 years or so. My immunologist put me on prednisone therapy, and I responded well. I am now on methotrexate, a drug also used for rheumatoid arthritis, and it seems to control my CSS well. Good luck.

  7. Thank you for telling your story, it has been really useful for me. I have been battling for the last 19 months with a cough which developed into pneumonia and then I was diagnosed with asthma. Up until this point I was A1 fit no complaints at all. Last year I was on and off steroids and my condition worsened to the point that I developed a temperature of 41 degrees, a skin rash which looked very red similar to chicken pox, flu-like sysmptoms which I had complained about for a year and general lethargy. I was admitted into hosptial in early December 2006 where it was found I was hyper eosinophilic. The doctors are finding it difficult to diagnose Churg Strauss but I am still having a lot of investigation work done. I will keep you informed of the outcome.

  8. i am a 57 year old woman who got a mild case of churg-struass last october. (I have asthma…I switched asthma meds from advair 500 to floradil last may) my dr. noticed a “funny” thing going on in my white blood cells. A blood test was ordered and the esinophillia count was 70% (norm is about 11%) prednisone was the only cure for me. My first symptoms were loss of appetite, numbness in left foot and sever pain in the left foot when waking in the night and getting up in the morn. I had swelling of the fluid around my heart and lungs causing me to have pain in my upper back. I lost about 10 lbs. it was hard for me to breathe. after many many test I was diagnosed with CSS. I have not had a problem since I stopped the prednisone last may… Just wonder if it can come back.
    Carolyn

  9. My Father is also suffring from this disease. Now, swelling in his feet as well as in his mouth is a huge problem for his health. He ia also problem with the weather condition…..

  10. I am terrified right now , and as I read this blog im trying to think back and remember all the things that have happened to me. My journey is ongoing but started about 5 or 6 years ago that I can remember. My doctors dont know what I have, they have classed me with sjogrens/ raynaunds / UCTD/vascullitis/autoimmune pancreatitis. As well as having Ulcerative colitis and thyroid disease since teens . Now I am thinking CSS, my eosiniphils have been climbing up to 8.90 now from 5.20 last bloods . I have had EMG and nerve conduction studies that show widespread neurapathy damage in feet lower legs and hands and fingers, as well as muscle weakness.

    I also get capillary bleeds in my finger tips and terrible abdominal pain esp a few hours after eating. This year I had trouble with breathing felt like I was drowning in air if that makes sense. So they did a xray and a pulmo fuction test. Lungs have some kind of looks like fiberglass to me in the right one, and they are hyperinflated. So on an inhaler I went, its still there and my doctors dont seem to know why. I did have really horrible pneumonia about 12 years ago almost died from it. Since then lots of chest infections.

    So with the latest news about the eosiniphils rising steady I started researching myself. I think in 2 weeks when I go to see my rheumy I am going to ask for the ANCA test. And bring this up, she said there was problems with my igG numbers and I think I read that also occurs in CSS.

    I live in a very rural area, and unfortunately I cannot drive, and live alone. If this is CSS I am terrified of what my future will hold. Thank you for the information, I see many things like GI bleeds when my UC is in remission that the docs cant explain.

  11. Hi James, thanks for all the information. It’s really helpful

    Hi everyone. I’m 27 and have been diagnosed with chrugs struass syndrome around 2 months back.

    A Little medical history of my case could be of some help to others. I have been sick from past 10 years witho weakness, sinusities, allergies, regular shortness of breath, fluctuating hyper and hypo thyrodism and severe cough and cold. I have sever bowel problem and acidity issues which was similar to appendicitis pain and had got my appendix removed for it. Doctors found lymph nodes elarged and upon biopsy, it was confirmed as lymphadenetis tuberclosis. This was around 4 years back which affected my immunity system and the medications reduced the bone density and vitamin D levels to a drastically threatening level. I have been falling sick every now and then and have been checking with doctors on it. None of the doctors could find the reason of my illness. It was during the last time when I have spiraliting joint pains and the medical reports revealed as rheumatic fever. Upon hospitalization, the diagnosed was confirmed as CSS. I was happy as there was a name to my condition but was sad knowing that its not curable.

    As my symptoms are mild, I have not been put on immuno suppresants rather on RA meds i.e hydrochroloqinione 200mcg for my joint paints. However, I have episodes of giddiness and fatigue on everyday basis which disrupts normal life. Doctors are keeping a watch and would put me on immuno suppressants if the symptoms aggrevates

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