Non Systemic Vasculitic Neuropathy, mononeuritis multiplex.

[stevoj]

Hi, I suffer with the above. Anybody else in the group with this? Anyone in East Anglia ?

[Hidden]

When you say non systemic, where is it, what has caused the inflammation and how do you know it is non systemic? What tests have you had? What have you been prescribed?

[stevoj in reply to Hidden]

Neuropathy affecting several nerves in both upper limbs (radial, median, suprascapular, C6 root), and 6th cranial nerve. Non- systemic so far. I've had numerous MRI, PET and CT scans, several LPs, radial nerve biopsy, so many blood tests I can't know them all, bone marrow biopsy. Probably missed a few tests, can't remember them all. On prednisolone and cyclophosphamide pulses. Diagnosis is likely vasculitis. As for the cause, that's a mystery.

[Hidden]

vasculitis.org.uk/about/loc...

Hi stevoj,

My reading of your post was that you have Vasculitus confined to your peripheral nervous system which is causing mononeuritis multiplex, or at least that's what the Dr's think it is.

You gave certainly had a battery of tests done, is the cyclo and prednisolone helping your symptoms? Sometimes it can take quite a while for nerves to regenerate.

I have posted a link to the VUK support groups so you can check if there is one near you. You are always welcome to phone or e mail the VUK helpline if you want to talk over aspects of your care and diagnosis.

Off the top of my head I would say that your type of Vasculitis is rare, are you treated by a Consultant who has experience in treating it?

Best wishes

Keyes

[stevoj in reply to Hidden]

Hi, thanks for your input and for the link. My consultant is extremely experienced in peripheral nerve disesse and in this condition. I was refered to her from my local Neurologists when they couldn't get a diagnosis. I agree, this is a very rare condition, with an unusual presentation. By the way, there has been no laboratory confirmation of vasculitis, which obviously does leave doubt as to the diagnosis. I've had no benefit from the treatment so far (8/10 treatments), in fact, I've had 2 further minor 'attacks' since being on the cyclo, so further room for doubt in the diagnosis methinks. But in the absence of any other diagnosis, this is what we're going with.

[Hidden in reply to stevoj]

It must be worrying Steve, have you ever had IVIG infusions? I don't know much about them other than they are sometimes useful for peripheral nerve problems.

If you have never seen a Dr who specialises in Vasculitis maybe your current Dr could liase with one, 2 heads are often better than one especially when symptoms are complex and diagnosis can't be confirmed.

Please do get in touch with the helpline if you feel you need to talk things over.

[stevoj]

No IVIG infusions yet, although lack of response to current treatment might suggest that this may be an option now.

[Hidden]

I have something rather similar going on although I do have signs of a systemic process from a lumbar puncture taken in January (paired O bands) and inflammatory markers that fluctuate dramatically so I suppose mine is probably systemic. But so far I've struggled to get a neurologist or rheumatologist to acknowledge that anything more is going on than parasthesia relating to a diagnoses of seronegative RA. I'm minded that mine might be some form of Sjogrens but had punch biopsies done and nerve conduction tests and no sign of damage to small nerve fibres has shown up yet. To complicate things further I'm highly allergic and have had severe reactions to four different disease modifying drugs to date. Currently slowly tapering off Prednisolone and under investigation by a new rheumatologist. I wish I could try IViG but so far this has never been suggested as an option. I think I have autonomic neuropathy but there seems to be no way of testing for this here in Scotland - or none that I've been made aware of anyhow. I will watch out for your progress with interest.

[AllanD in reply to Hidden]

I can see that these posts are 2 years old and I am wondering if any progress has been made. I have also been identified with non systemic peripheral nerve vasculitis but in my case, vasculitis was confirmed from a Sural nerve biopsy. Also interested to hear about any consultants in Scotland with experience in treating non systemic peripheral nerve vasculitis.

[Hidden]

Hi AllanD,

I’m sorry that you’ve had similar problems to Stevoj. It would indeed by useful to know the outcome, if there has been one.

Personally I’m still struggling with neuropathy - new non systemic pins and needles the length of both arms with bloods all being relatively normal now and no explanation yet.

The neuropathy has caused permanent damage now everywhere (loss of sensation causing mobility and balance problems) and I wish I’d been offered IViG a few years ago when things were at their worst pain wise.

I did get rediagnosed from RA to Sjögren’s by lip biopsy and high ANA last year. I’ve been on Mycophenolate for a year but it has only helped with the systemic stuff and arthralgia so far - not the neuro symptoms, which appear to be progressing.

I’m in north Fife under neurology and Rheumatology in Dundee and have just seen my neuro a few weeks ago - but she’s never suggested a nerve biopsy yet or repeated imaging or confirmed small fibre and ganglionopathy diagnosis so far. Everything gets blamed on my confirmed Sjögren’s now but I’m really not so sure myself. Seeing my rheumatogist again next week.

[AllanD in reply to Hidden]

My blood tests all come back normal too. Not a good indicator for this problem.

For me, medication is a reducing dose of Prednisolone and Methotrexate. Been on the Methotrexate for just under 3 months but still no signs of improvement and more sensory loss identified.

The path to diagnosis for me was through nerve conduction tests which confirmed a problem with the nerves, then the biopsy which displayed vasculitis in the nerve and allowed my Neurologist to put a name on my condition. Maybe the nerve conduction tests are the next step for you. The nerve biopsy has caused pain and discomfort ever since the procedure was completed but I am hoping that this will fade with time. Not something to go through if it can be avoided and it does not provide a cure, only confirmation of the condition.

Hopefully your rheumatogist will be able to provide more answers next week.

[Hidden in reply to AllanD]

Thanks AlanD. My nerve conduction studies (a year ago and 3 years ago) have been entirely normal so far - which is in keeping with a non length dependent small fibre neuropathy. My lip biopsy was 100% positive of Sjögren’s so this is what I have. But Sjögren’s is often associated with certain types of Vasculitis and - as the pins and needles and loss of sensation everywhere progress and my inflammation markers continue to fluctuate between elevated and high - I’m really starting to wonder. For me I think it’s more of an immune mediated, inflammatory neuropathy, than the self limiting, length dependent SFN suffered by many with Sjögren’s.

I’m on the maximum dose of Mycophenolate now but have tried Methotrexate and Prednisolone and many others in the past.

Have you been offered IViG yet?