I was diagnosed with Cranial Ateritis in 2011 by my GP and quickly saw a consultant who changed that to Vasculitis. Over the following year or so that was changed again to Wegener's and also again to include brain (lining) involvement resulting in partial failure of my right cranial nerves. The disease proceeded in the almost normal way.... right side headache, sudden deafness in right ear, sinus problems, facial and ear pain and then a suspected TIA resulting in the right vocal cord being paralysed, tongue deviation and some general loss of control on my right side. Apart from the vocal cord, almost all back to normal now. The disease only affected my right side.... you could draw a line from my forehead through my nose to my groin and the only problems are on the right side. I had the full range of scans etc.
I also have 2 hearing aids... my left ear is due to age related deafness and my right ear to nerve deafness. BUT I am not complaining.... I feel sort of alright.... aches and niggles and all the other things that Wegener's brings on; I am an optimist and very rarely feel down.
About two months ago I began to get left sided headaches and realised that they were not just normal headaches but reminiscent of the earlier right sided ones I got before. I also had some congestion in my left lung. Then my hearing in the left ear diminished along with an increase in pain, and at that point I rang the Rheumatology dept.... I got in quickly ... yesterday to be exact, and I was seen by a delightful young lady doctor whom I have to say was very clued up on the subject. I read through my bulleted notes to her and she examined me and interrogated me very thoroughly. She then summoned 'God' (Prof Emery) chief consultant, who also checked me over and questioned me.
The result of all this is that they feel the Wegener's is moving to include the left side of my body, which is what I thought was happening, and although it is good in a way to be proved right, it's also bad news. I am dreading the further loss of hearing in my left ear. My next infusion of Rituximab will be brought forward, probably within the next fortnight, and I have to have a new round of tests. My steroids have been increased from 10mg to 40mg with a progressive taper after Rituximab.
All this just when I thought I was getting used it and feeling fairly good. Don't get me wrong, I don't feel ill or nauseated, just a bit pi55ed off.
I am in Leeds and being seen at Chapel Allerton hospital. The team there is A1... you couldn't ask for better... they talk to you (not at you) and they listen to you. They take your opinions to heart and spend time explaining what their plans are for you. I even had a phone call from the young lady doctor this afternoon asking to clarify a point.
Yet another step in this big adventure.
Apologies... I don't post often but when I do it is usually lengthy.
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lfu2
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You do sound as if you are a complex case. WG can vary so much from person to person ... How long have you been on Rituximab and what intervals? Do you take any maintenance medication alongside the RTX. We do know that the team at Leeds is good.
This will be my 5th cycle. I was on 6 month cycles to begin with and then to annual cycles. I also take 20mg methotrexate; along with a plethora of other lesser meds. Thank you for the interest.
Hi, I just read your post and thought to myself how could Wegners only affect one side of your body? I don't mean to offend but is that not impossible? I know it can affect different parts of the body but I have never heard of anybody having it one side only?
My mum had a similar experience to you, vasculitis causing tia we suspect but without exhaustive scans and tests we will never know, mum didn't want that and her treatment would have been the same.
Her tia and vasculitis caused problems "evenly" for want of a better term throughout her body.
Have you been scanned to make sure another tia isn't causing this?
Hi Luggsey.... an interesting question. The neurologist couldn't give me a proper answer as this was a first for him as well. With hindsight I think the infection was all over my body but was seen best/worst only in the places where the TIA had affected me. He said the brain lining was compressed on the right side thus compromising the 12 cranial nerves which exit through the meninges and consequently only affected the right side of my body. I have had numerous scans including 2 brain MRI's which showed the development of the compression. More scans are being booked.
An interesting neurological addition to the diagnosis was neuro sarcoidosis which could explain the one sidedness of the wegener's (so I have been informed).
Very interesting! There is a Neuro in London who has an interest in Cerebral Vasculitis and is a Neuro Sarcoidosis expert as wel. It might be worth asking your current team to run your symptoms and scans past him to see if he has any insight to offer. A collaborative approach is essential to get to the bottom of rare, complex symptoms.
Hi - actually I've come back to this community through Ifu's NRAS HU post mistake - because the contents of this post itself intrigued me.
Regarding the one sided matter - I'm very interested in this having had a small fibre neuropathy which affects all four limbs and my face - but only the left side above the mouth.
Last year I suffered three stroke-like episodes - later described as left sided functional hemiparesis episodes, following extreme pain/ sepsis and having taken Tramadol. However the mild numbness, tingle and twitching has affected only my left nostril, cheek and eye to date. The rest of my neuropathy is bilateral but I also have been suffering from tinnitus recently - mainly in my left ear I think.
I don't have a diagnosis of Vasculitis, was diagnosed with RA five years ago, but now have a definitive diagnosis of Sjogren's (lip biopsy with high amounts of lymphocytes and positive ANA plus other positive immunology and high CRP and PV all showed this). I see the consultant this coming week and expect to be rediagnosed at last. It has taken a fourth rheumatologist whom I haven't yet met - to work this out.
Vasculitis is the one disease that doctors consistently mention as a possibility. But actually my ANA shows positive for Scleroderma. Nothing dull about my health anyway!
Sorry to hear this, can completely empathise, best of luck in your journey, you have a right to be pissed off, but it's also clear you're in a good frame of mind. Stay peaceful. All the best
Thank you for your story. I became acutely in 2012 after developing chest problems over the previous few years, which progressed from inhalers yo needing regular 30mg prednisone be daily.
In November 2011 I developed sudden deafness in my right ear followed 2 weeks later by awful temporal pain which radiated down the side of my face in front of my right ear into my jaw. I was diagnosed with an ear infection by my GP.
To cut a long story short in March 2012 after 3 A an E emergency visits for extreme breathlessness and congestion I was admitted to hospital. I had a collapsed lung and pseudomonas was grown in the sputum.
I was in my local hospital 4 times between March and July - at least 2 weeks each time - on IV Tazocin (antibiotic) and on the last occasion Meropenem.
These powerful antibiotics should be given at the specified times and never more closely than 4 hours. The time I was given Tazocin depended on which staff were on duty. On one occasion at 11.50 am I was still reminding the 'sister' who was supposed to admister my medication during her shift that I had not had my 8am Tazocin. She then administered my next dose before 4 hours had elapsed. It is no wonder I developed C. Diff!
The very senior Professor of Rheumatology at the hospital decided I had 'enhanced pain syndrome' and needed Amytriphilline - i.e. nothing physically wrong with me.
Both my daughters have SLE ( Lupus).
After discharge I got copies of my hospital records which showed My CRP (an inflammatory marker in the blood) was 192 on more than one occasion my ESR also very high. These blood test results stated that such high results suggested acute INFLAMATION or infection. As I was not responding to the antibiotics it is surprising an inflammatory diagnosis was not considered especially as I also had a positive C Anca blood test result. One junior doctor in A and E who examined me during my first admission actually diagnosed Vasculitis but as on admission I was taking a daily dose of 20 mg because of asthma a senior doctor over ruled this.
Once home I did as much research as I could and will always be grateful to Vasculitis UK for their support when I first came out of hospital.
I am now under a wonderful hospital where patients are treated with dignity and respect. Every doctor I have seen has had a holistic approach.
Since attending this wonder facility my bloods have not shown anything dramatic, no positive C Anca. ESR seldom raised. I have had recurrent GCA and polymyalgia which rescures when reducing steroids. I also take 20 mgs Methotrexate in addition to what I am sure is the usual cocktail of medication.
I have inflammatory arthritis in my hands and in addition to problems caused by Vasculitis. My fingers and thumbs, especially the pads become terribly red and painful and swell. This can go on for a couple of weeks. It is agony to touch anything. The last time this happened I was reducing prednisolone and was down to 15 mgs daily. On the increased dose to 40 mgs daily the swelling and pain began to subside. Then white areas developed in the skin and over the next two weeks the skin sloughed / peeled off. I took pictures which are not pleasant but are good to have to be able to show the progression.
i am still waiting confirmation of what sort of Vasculitis I have. It has been confirmed my hearing loss is due to nerve damage I wear two hearing aids. My lungs are damaged I have have been diagnosed with bronchieactasis and COPD. During the last episode of GCA I had red/purple rashes on the shins of both legs. But with pretty ordinary blood results I can understand the doctors may have a dilemma.
I am worried about my memory and concentration and the total exhaustion I often experience.
If anyone has experienced similiar difficulties early in their illness I hope my story may be of some benefit to them.
Churg Strauss was never considered as I have never had raised esinophyll levels. When I had a second GCA attack my Temporal artery was scanned and it was found to be 'occluded and engorged' at the area going up from in front of my right ear to forehead. I had a biopsy but not for 16 days post 60mgs of prednisolone for 5 days and then on 40mgs. the biopsy was negative but I had been told before having it that it might be. I was still under my local hospital at that time and an immediate quick reduction in steroids was ordered with no follow up.
In addition to SLE (Lupus) both my daughters have other auto-immune conditions, one has Crohn's the other Ulcerative Colitis and Reynaulds. I understand it is not uncommon to have more than one auto-immune problem.
I had a positive Shirmer test (if that is the right term for very dry eyes), I have virtually no saliva and have other symptoms. When I consider I have lung inflammation, deafness caused by nerve damage and recently during the last attack of PMR and GCA I experienced painful eye sockets with pain going down from the inner aspect of my left eye down my nose. 24 hours later the area around my left eye on the inner aspect adjacent to the nose was black - I had obviously had a bleed into that area.
I took photos of this as I do of all rashes, swollen fingers, etc to show Consultant as they may not be visible when I go to appointments, but with all this going on I am puzzled by my current normal blood tests.
I wonder if I have Wegners as well but this has not been mentioned.
I have been on high dose prednisolone since 2011,and I wonder if this could be the reason for the blood results, however at clinic I meet patients whose blood results are much worse that mine.
For over a year I have been on Methotrexate 20 mgs once a week, hydroxychloroquine 200mgs twice a day in addition to other common meds. Unfortunately because of chest infections I have not been able to take the Methotrexate every week (having to take antibiotics). I was slowly reducing my prednisolone dose from 40 mgs daily and had reduced to 10 and was stable Then I was told to reduce by one mg every couple of weeks and once I reduced to 8 everything went wrong. Everything flared.
I have been offered rituximab but am reluctant - I know the doctors are trying to help me and perhaps I am an unusual case where diagnosis overlaps or is difficult, but the treatment can be as bad as the illness.
I am so grateful for your reply. I used to such an active busy person leading a very full life while also supporting my daughters and their families. Since being so ill for so long I have become isolated. 'Friends' have dropped away.
I am unreliable as it is difficult to commit to anything in case I have to cancel.
I also suffer from osteoporosis - not only from steroid use - it has a possible genetic cause- and have 4 fractured vertebrae as well as disc problems. back pain is a constant problem.
However, please don't think I feel sorry for myself. I hide as much as I can from my daughters as I don't want them worrying about me and if the vasculitis is not too bad I almost forget I am ill and try and read and study. I believe a positive attitude is very important in life but also that knowledge is power. It would be easier if I had a definite diagnosis.
I can appreciate the complexity of your illness as I have a possible spondyloarthropathy/ Vasculitis overlap which presented with PMR/GCA type symptoms.
I am on biologic drugs ( Infliximab infusions ) which have made a big difference to my symptoms. Is there any particular reason you are reluctant to try Rituximab? It has been a wonder drug for many patients with Vasculitis, especially ANCA positive types such as Wegners. The likes of prednisolone is far more toxic in the long term especially with your oesteoporosis.
It might be worthwhile posting to ask for patients experiences of Rituximab, many people on here have had it and are always willing to share their stories.
I think a lot of us can appreciate the difficulties of having a rare, chronic, long term illness. You don't sound in the least sorry for yourself. The great thing about this group is that we understand.
In 2011 I developed Steven Johnson Syndrome from the Osteoporosis drug Strontium Ranelate. It was the most terrible experience, it was 4 months before I was recovered and huge doses of steroids and other drugs were needed. It was a ghastly painful time.
I read as much as I could about rituximab and noted references to Steven Johnson Syndrome and PML. Having also developed C.Diff (on three separate occasions) from the I.V. antibiotics I had been given when first ill. I thought that if anyone would be a candidate for the PML complication it would be me.
Since the Steven Johnson experience I have developed drug intolerance that I did not have before. I also noted research that seemed to indicate that 60% of patients benefited from rituximab, which I felt was not high enough to warrant the risk given my history.
I understand other biologic therapies do not have the PML risk but these have not been suggested but I will ask the Consultant why rituximab if the preferred choice for me.
To be honest I don't think I will have much choice. Before my last 'flare' I was reducing prednisolone by one mg every month but on my last visit (when I declined rituximab) I have been told to reduce by 5 mgs every two weeks. Past experience suggests to me that I will be very ill quite quickly.
Thank you so much for your reply. It is very encouraging to know that you have had benefit from a biologic treatment and I am very grateful.
I have nursed patients with severe Steven Johnson syndrome in ITU so can appreciate what a truly horrible experience it is. I was aware of the link between Rituximab and PML, interestingly I wasn't able to find a case involving Vasculitis, just RA and SLE.
These are scary decisions to make, in a way Vasculitis is a double whammy, not only is the disease life threatening but the side effects of treatment can be as well. I hope you manage to find a drug regime that works for you but carries a low a risk as possible.
That's a hell of a story... it just makes me realise how easy my diagnosis and my journey has been. I can only wish you luck and better health. You deserve a gold medal for just carrying on with your life. I hope you get a definitive diagnosis soon. I will be thinking of you.
Thank you so much for your kind reply. For the first time for very long time I don't feel so isolated.
Your post of 2 days ago was the first I read since joining HealthUnlocked. Thank you for your story I do hope you keep well. I have huge empathy with the emotions you expressed - hope you share some more!
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What is your experience of Rituximab infusions for Wegeners?
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