[MAT52]

I'm hoping no news is good news this time. Haven't updated here since my rheumatology appointment nearly weeks ago. As expected I was rediagnosed from 2011 diagnosis of seronegative RA to primary Sjogren's. It was a little more daunting than what I was expecting because he then went on to say "without wishing to frighten you more than necessary" - that he was concerned my neuro symptoms were showing CNS involvement as part of the Sjogren's. I had a brain MRI in a different hospital 18 months ago and was told all was normal despite some white matter signifying small vessel disease.

The rheumatologist has written to the neurologist (who told me I was overthinking and looked too well to have a multisystem disease back in April) asking her to study my previous MRI and compare it with a new one I am getting done tomorrow. His hunch is that the SVD is part of my Sjogren's and if she concurs with this then he thinks I should try a big gun drug such as Rituximab.

He also sent me for a CT with contrast of thorax down to pelvis to rule out Sjogrens associated Lymphoma because of other symptoms and a swollen lymph node in my groin. I had this done a week ago and have heard nothing yet, so cautiously assuming that no news is good news - although this is the beleaguered NHS so I will phone hospital in a few days and clarify. Fingers crossed!

I'm feeling strangely calm and optimistic about all this now thanks to friends on the Sjogren's World Forum,Lupus UK healthunlocked and the Brittish Sjogren's Syndrome Association forum. I guess I also just feel SO much better to have a diagnosis I believe and can attribute most, if not all of my symptoms at last. I'm not expecting either test to show up anything too scary. And even if it does I'm just too sick of worrying after five years of feeling wrongly diagnosed and always up against it. It's relative chill time just now.

However I will always feel the greatest about of sympathy for those who are unable to find a proper reason for their symptoms or enough signs be properly diagnosed or treated. The shadowlands are no place to dwell for too long. Even waiting for results is less awful now there's a disease name attached at last. Courage and perseverence to all shadowlanders here.

If anyone else here suffers from disequilbrium/ vertigo as a result of small vessel disease in the brain then it would be great to hear from you about how it feels. Regarding my small fibre neuropathy (legs, arms and mouth) where has mine gone? I know about the SFN causing propriception problems and am thinking mine is a classic case of these peripheral issues too. But what I'm not quite sure about is whether the SFN is severe enough to have caused this severity of disequilbrium? The rheumatologist calls it "presumed SFN" because it didn't show up in skin biopsies taken from each calf last year. I admit that I just can't tell if the neuropathy is bad enough to cause my quite severe disequilbrium because it's stopped hurting much for a while now. It still does tingle and I do still have vague crawling sensations and occasional flash pains in my hands. But it's really nothing to moan about- not the excruciating symptom that paralysed me each night this time last year. So have many of my tiny nerves died causing some numbness as the neuro thought or has the SFN simply subsided? How can I tell? More importantly how can a neurologist tell without biopsying me again - this time properly?

[en bloc]

Small vessel ischemic disease in the brain does NOT typically cause vertigo as you describe. Actually, most people don't have any symptoms unless it is wide spread or significant in size/amount of the ischemia (which yours did not indicate). The only other symptoms typically reported with SVID is cognitive type problems with thinking, memory, etc...which you haven't said you experience. Again, this would only be normally seen with significant SVID, not mild.

So hopefully this will ease your mind about the significance of this finding. I can say that the SVID may be from the Sjogren's...I have pretty significant SVID as a result of my Sjogren's...but I also have APS and have had 3 strokes and numerous TIA's.

It will be very interesting to see if there is any changes in your MRI from 18 months ago and now...that is what will tell whether your changes (vertigo) are consistent with any disease process that has progressed. If I remember correctly, you didn't have the vertigo back when the first MRI was done.

You really need another biopsy. I thought you had positive findings for SFN on the first. If it was negative, then by all means it should be repeated. And most importantly, is to have the morphology done on the next skin biopsy. One significant finding with Sjogren's related SFN is that the fiber density can be normal but the morphology (condition of the fibers) show damage. My density was normal on the first biopsy, but the damage was very clear with segmented, torturous, and swollen fibers in many areas. This is what Sjogren's can do to the fibers...not necessarily only reducing density. And this finding is just, if not more, important. You also need to have more than one location done on each leg. There is NO way to determine length or non-length dependent SFN if only one site is biopsied on the each leg.

Hope this helps. Please keep us posted.

[bluesfan]

Mat

Thanks for getting back to us - had been wondering how you were getting on.
As bad as it may sound to say "well done" on getting your Sjogren's diagnosis, it was your perseverance, determination and conviction that you knew something was wrong, that got you there. I hope you feel even a small sense of victory at having taken on the NHS system (with all it's faults), and triumphed over all the naysayers you had to put up with along the way. If nothing else the experience will have given you great skills to manage your own health outcomes in the future.

I can empathize on the sense of relief at finally having a definitive diagnosis after so many years. My father had Sjogren's for many years and it's something I've been tested for (and will continue to be as I already have another auto-immune condition). I wish you all the best for the forthcoming tests and it's good to hear you're getting lots of support from others with the condition.

[MAT52]

Originally Posted by en bloc

Small vessel ischemic disease in the brain does NOT typically cause vertigo as you describe. Actually, most people don't have any symptoms unless it is wide spread or significant in size/amount of the ischemia (which yours did not indicate). The only other symptoms typically reported with SVID is cognitive type problems with thinking, memory, etc...which you haven't said you experience. Again, this would only be normally seen with significant SVID, not mild.

So hopefully this will ease your mind about the significance of this finding. I can say that the SVID may be from the Sjogren's...I have pretty significant SVID as a result of my Sjogren's...but I also have APS and have had 3 strokes and numerous TIA's.

It will be very interesting to see if there is any changes in your MRI from 18 months ago and now...that is what will tell whether your changes (vertigo) are consistent with any disease process that has progressed. If I remember correctly, you didn't have the vertigo back when the first MRI was done.

You really need another biopsy. I thought you had positive findings for SFN on the first. If it was negative, then by all means it should be repeated. And most importantly, is to have the morphology done on the next skin biopsy. One significant finding with Sjogren's related SFN is that the fiber density can be normal but the morphology (condition of the fibers) show damage. My density was normal on the first biopsy, but the damage was very clear with segmented, torturous, and swollen fibers in many areas. This is what Sjogren's can do to the fibers...not necessarily only reducing density. And this finding is just, if not more, important. You also need to have more than one location done on each leg. There is NO way to determine length or non-length dependent SFN if only one site is biopsied on the each leg.

Hope this helps. Please keep us posted.

Thanks Enbloc - this is all in line with what I suspected so am grateful with you for both confirming and reassuring me. I absolutely hate the thought of vascular dementia because both my parents had this in the run up to their untimely deaths from cardiac arrest. But you are right to say that I have no signs of cognitive impairment yet. Just this wretched vertigo/ disequilbrium. I actually did have it last year too around the period of time the MRI was done but it was more intermittent and I didn't have it that actual week or month. However I had it a few months before when I went to see the neurologist for the first time so it was on the menu so to speak!

I'm not at all confident that my skin biopsies were taken correctly since my GP offered to do them when I was ill with pneumonia. He was a little pushy as had never done this before and wanted the experience. He followed the neuro pathologist's instructions and took one punch biopsy from the outer side of each calf. Which makes me think that neither knew what to do properly! I may have to push to be sent to London for it although the big hospital I'm with now should be much better placed to know what to do.

I know you are spot on because there is an article about it in an edition I have of the Brittish Sjogren's Syndrome Association magazine from earlier this year - in an edition dedicated to SFN and ganglionopathy of Sjogrens - showing photos of where the biopsy should be taken from. There seems to only be one from each leg though but these are far closer to the ankle than mine were. I'm also not that confident that the samples were stored and dispatched at the right temperature seeing as they were taken while on my island home and send to two different hospitals.

So my rheumatologist refers to my SFN as presumed rather than clinically diagnosed. This is why I'm not that confident that I will qualify for any further immunesuppression such as Cellcept if there is no organ involvement showing up from my CT/ MRI. I will try to discuss this with my neurologist next month.

I know you are very much further down the line than me with symptoms and signs, but out of interest had your SFN turned from being severely painful to tingly and somewhat numb by the time your second biopsies were taken? I'm just not that confident that my symptoms show a worsening, although I do believe that pain is an indication that the tiny nerves haven't yet died? So my lack of pain in legs and feet now may signify worsening rather than improvement? The burning pins and needles pain is worse in my hands and arms than in my other extremities, but really not too bad at all compared to this time last year.

[MAT52]

Originally Posted by bluesfan

Mat

Thanks for getting back to us - had been wondering how you were getting on.
As bad as it may sound to say "well done" on getting your Sjogren's diagnosis, it was your perseverance, determination and conviction that you knew something was wrong, that got you there. I hope you feel even a small sense of victory at having taken on the NHS system (with all it's faults), and triumphed over all the naysayers you had to put up with along the way. If nothing else the experience will have given you great skills to manage your own health outcomes in the future.

I can empathize on the sense of relief at finally having a definitive diagnosis after so many years. My father had Sjogren's for many years and it's something I've been tested for (and will continue to be as I already have another auto-immune condition). I wish you all the best for the forthcoming tests and it's good to hear you're getting lots of support from others with the condition.

Thanks Bluesfan - yes I absolutely do feel vindicated you are right! I wrote a post on Lupus UK forum headed "vindication by lip biopsy" and this expresses my sense of triumph I think.

However the reality is that primary Sjogren's (clinically diagnosed that is) is listed as a rare disease here in the UK and very under recognised and misunderstood compared to the other connective tissue diseases compared to the USA and other countries. The NHS SS page makes no mention of SFN/ PN or neuro symptoms or drug treatment options. There is, I have observed, a newly formed alliance of rare rheumatic diseases in the UK. It includes Lupus, Scleroderma and Vasculitis but no mention of primary Sjogren's.

It appears that even when it's properly recognised/ diagnosed doctors think only of the dryness of eyes, mouth and vagina. This seems to be the extent of what many understand and the neuro symptoms require a specialist SS expert. I say this with confidence now that three rheumatologists and two neurologists have failed to identify mine - even with the sicca listed on my sheet of diagnosed conditions. It took my ANA switching from negative to positive to get the new chap to turn blood hound for me. And sadly he's retiring from practice this month.

So if I'm to qualify for anything other than topical treatments only (and I know these are very important of course) then I need to feel confident in neurologist and rheumatologist working together to find answers and treat the disease at source. Way still to go but extremely grateful for your support as ever. It's taken me longer to come here and report my news to date because I really wanted to have something more to ask and say than just a rather euphoric splurge! X

[en bloc]

My SFN had increased in pain by the time the repeat biopsy was done (hence why we did it). The density was decreased from the first but more importantly, the condition of the fibers was once again showing clear damage...at every location (and I had 6 locations done--three sites on each leg---ankle, just above the knee and upper thigh). So the damage was wide-spread, typical with Sjogren's.

I have heard people here say that their pain turned to numbness, but most often I think it's just increased pain as the SFN worsens. The repeat biopsy would show definitively what you have going on...especially if they do morphology testing.

[MAT52]

Originally Posted by en bloc

My SFN had increased in pain by the time the repeat biopsy was done (hence why we did it). The density was decreased from the first but more importantly, the condition of the fibers was once again showing clear damage...at every location (and I had 6 locations done--three sites on each leg---ankle, just above the knee and upper thigh). So the damage was wide-spread, typical with Sjogren's.

I have heard people here say that their pain turned to numbness, but most often I think it's just increased pain as the SFN worsens. The repeat biopsy would show definitively what you have going on...especially if they do morphology testing.

Thanks for describing your own experience. I must say I think mine peaked last year because I recall, following surgery for gallbladder, being in so much pain in my feet, legs and hands afterwards, that I barely noticed the localised pain of the operation site. The nurse was baffled by me as I kept crying out about my peripheries. "It must be some kind of bizarre referred pain?" one of them said to her colleague.

So now this stinging/ burning in my hands and vaguely numb sensation in feet just doesn't seem worthy of digging more wee holes out of myself for. I suppose I just think I should be very thankful rather than push for further tests. Plus I found the anaesthetic sites excruciating. But then each of us has our own unique response to pain so it could just be this. My GP took each of my biopsies from the area of my upper calves where I felt the least pain, just as the neuro pathologist suggested he do after tapping my calves with a nerve conduction pin.

[en bloc]

I understand your thinking on this, but you might want to repeat the biopsy if they are considering any big guns. Knowing if you truly have SFN (and the extent of damage) would be critical info before even thinking of taking IVIG or Rituximab. One important factor to consider is having a baseline (which you really don't have since the biopsy wasn't done at the proper location or in more than one site...not to mention your question of handling/transport, etc.) Once you start a big gun treatment you will never know the original amount of damage and/or if the treatment is working. This is one of the great benefits of this test is that is is repeatable (many times) to gauge progression of disease process or documenting improvement so you know whether to continue treatment. And of course, the test is very minimally invasive with just tiny punches of skin being removed and simple bandaids for dressing.

Also, I can't image they would start a big gun if you didn't have confirmed SFN from the Sjogren's. They don't use Rituximab or IVIG for SVID or autonomic dysfunction. And I don't think you have said you have any organ involvement either (other reason to use these big guns). So confirming the SFN is really important if they still want to even think of a big gun treatment.

Just food for thought.

[MAT52]

Originally Posted by en bloc

I understand your thinking on this, but you might want to repeat the biopsy if they are considering any big guns. Knowing if you truly have SFN (and the extent of damage) would be critical info before even thinking of taking IVIG or Rituximab. One important factor to consider is having a baseline (which you really don't have since the biopsy wasn't done at the proper location or in more than one site...not to mention your question of handling/transport, etc.) Once you start a big gun treatment you will never know the original amount of damage and/or if the treatment is working. This is one of the great benefits of this test is that is is repeatable (many times) to gauge progression of disease process or documenting improvement so you know whether to continue treatment. And of course, the test is very minimally invasive with just tiny punches of skin being removed and simple bandaids for dressing.

Also, I can't image they would start a big gun if you didn't have confirmed SFN from the Sjogren's. They don't use Rituximab or IVIG for SVID or autonomic dysfunction. And I don't think you have said you have any organ involvement either (other reason to use these big guns). So confirming the SFN is really important if they still want to even think of a big gun treatment.

Just food for thought.

Yes - I have only just begun to think along these lines myself Enbloc but you may well be right.

My rheumatologist hasn't mentioned this option though. He seems to be thinking that progress of SVID will warrant a big gun of itself. In his clinical letter he is asking the neurologist to compare the two MRI scans and tell him whether these show progressive SVID which is in keeping with Sjogren's as he suspects - in which case he feels a further immunesupnpresant or a big gun is warranted. So his questions of neuro are two fold - have I got white matter showing SVID? And is the white matter sufficiently progressing in line with my Sjogren's? If answer to both is yes then he or his successor should consider these. IViG is not mentioned.

The CT a week ago was to look for other organ involvement. I take it that this showed none as I haven't heard anything yet but the MRI nurse said she wondered if he was trying to get all the info he's requested on his desk so he can look at the bigger picture. So not 100% sure about this either.

My feeling is that if nothing shows up that makes further immunesuppressants available to me I will ask about getting punch biopsies done. But last night I looked to see where UK Sjogrens experts were who might commission these biopsies. And this research confirmed that there is very little expertise for SS in the UK. The few that exist are in Newcastle and London. So your experience of investigations and treatment for SS is going to be very different to mine. I can't afford to seek this expertise out privately so have to hope that the knowledge is in this large university teaching Hospital - but just hasn't shown up on the expertise list yet.

To my dismay I found that there is a new alliance of rare rheumatic disease organisations in the UK and Sjogren's isn't even listed. This shows how far we have to go here in the UK I feel. It seems to be way behind other countries and on the NHS Sjogren's page neuropathy isn't even referred to. On the Patient.com page it says everyone with Sjogren's will have dry mouth and eyes. And yet in the BSSA magazine it says that some will present with neurological symptoms long before the disease manifests significantly in mouth or eyes.

[MAT52]

Originally Posted by en bloc

I understand your thinking on this, but you might want to repeat the biopsy if they are considering any big guns. Knowing if you truly have SFN (and the extent of damage) would be critical info before even thinking of taking IVIG or Rituximab. One important factor to consider is having a baseline (which you really don't have since the biopsy wasn't done at the proper location or in more than one site...not to mention your question of handling/transport, etc.) Once you start a big gun treatment you will never know the original amount of damage and/or if the treatment is working. This is one of the great benefits of this test is that is is repeatable (many times) to gauge progression of disease process or documenting improvement so you know whether to continue treatment. And of course, the test is very minimally invasive with just tiny punches of skin being removed and simple bandaids for dressing.

Also, I can't image they would start a big gun if you didn't have confirmed SFN from the Sjogren's. They don't use Rituximab or IVIG for SVID or autonomic dysfunction. And I don't think you have said you have any organ involvement either (other reason to use these big guns). So confirming the SFN is really important if they still want to even think of a big gun treatment.

Just food for thought.

Ps further to my earlier reply - I've recalled that my rheumy says that SFN of Sjogren's usually only goes so far and then stops and neither improves nor progresses - it is something sufferers just have to learn to live with. So, unlike SVID, it is not treated with big gun or immunesupnpresant therapies even when it is confirmed. Almost the opposite of what you have been told I think?