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.