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

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.

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.

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?

As you said, your rheumy (and your area) doesn't seem to have the expertise on Sjogren's, and this may be clearly evident by his comment that SFN doesn't progress in those with Sjogren's (very wrong). Was this the old doctor that just retired? I doubt he is up on the latest with Sjogren's.

I'm hoping your large teaching hospital offers you more options and opinions.

There are numerous articles (that have been posted on this board as well as Sjogren's World) that document the neuro complications (including SFN) of Sjogren's. There is NO mention of non-progression or that it will cause certain amount of damage then no more and patients just have to live with it. Sorry but that just blows my mind. But I feel confident you will get other opinions from your new rheumy and the team at this teaching hospital.