For appointment on 12th October 2016

New Symptoms

•Occasional vision issues/ sudden blurring/ difficulty focussing

•Tinnitus (very high frequency whistle - left ear or both 24/7?) – over sensitivity to noise

•Uncontrollable body temperature swings – sweats (especially at night)

•Very itchy, stinging fingertips despite no sign of dry skin

•Severe pain with stiffness in knuckles – early hours of every night. Can't lift duvet or move pillows about without excruciating pain in little and index knuckles of both hands. Eases during the morning. Severe pins and needles in both hands and wrists.

Worsening or ongoing symptoms

•Dizziness/vertigo/ disequilibrium/ sensation of dropping into space when head lifts or turns/ out of kilter walking - trouble interpreting unfamiliar steps and patterned flooring/ artificial lighting all cause confusion.

• Weakness in hands, wrists, elbows, knees and ankles, especially while resting

• Pins and needles in hands, wrists, gums, lips and eyes and wet, skin crawling sensation on legs. Lips and gums feel permanently tight, sore, swollen.

•bone pain in feet and shins and arms

•Facial numbness now affecting eyes

•Bad taste 24/7 – (salt, vinegar, off milk)

•Frequent need to pee

•icy cold extremities – sometimes with colour changes, mainly toes with hard, powdery white or shiny skin

•Insomnia and Fatigue

•Constipation

Questions:

1.Could all the presumed small fibre neuropathy actually be Raynauds/ circulation related?

2.Could my dizziness, off kilter, disequilibrium, and tinnitus actually be inner ear related?

3. Could the cause of the pins and needles everywhere all be due to nerve entrapment from inflamed tissue (given my consistently very high PV/ ESR and raised CRP)

4. If you think my problems are neuropathic then would punch skin biopsies to assess for small fibre neuropathic nerve damage taken from each leg be a next appropriate step - given that this was last done by a GP (Scottish island) 18 months ago – who had never done this before and wasn't even sure where to take skin from or where samples would actually go or how they would be transported?

5. Could temperature swings, awful taste, constipation and dizziness be dysautonomia relating to my Sjogrens and hypothyroidism?

6 If tests confirm this - would treatment such as IViG be helpful in minimising the progress of these types of sensory neuropathy?

1) I sort of doubt you can assume these symptoms are Raynaud's. The color changes you mention are to white powdery/shiny...not the typical blue and red changes. And Raynaud's wouldn't explain the facial SFN symptoms.

2) Absolutely! If this hasn't been explored, then by all means, you should see an ENT.

3) Of course it could...to some extent. I quick steroid pulse would tell you whether inflammation is the culprit. Symptoms would greatly reduce in a matter of 2-3 days with the steroids. But again, this doesn't explain the facial symptoms. Only full body SFN would seem to explain the wide spread symptoms you have.

4) YES!! You know how I feel about this...I've been singing this tune for a while...LOL But it would be nice to see what Glenn thinks or others , as well.

5) Yes, even some eye/vision symptoms are a result of dysautonomia. I have frequent spells of double vision from autonomic dysfunction.

6) IVIG would be best bet to slow progression and reduce symptoms for long term gains. Sjogren's mediated neuropathy can cause extensive damage in a short period of time and should be treated immediately for best results. Dr. Birnbaum told me that aggressive treatment AFTER the first year shows less positive results. The other med that may be helpful is Cellcept. Steroids may also be an option...a pulse of steroids, to see if this is an inflammatory based neuropathy.

Being this neurologist's first visit and blowing you off, you should focus on the increase/worsening of symptoms and especially the new symptoms. The confirmation of your Sjogren's Dx should open her eyes have her start singing a NEW tune. She needs to start treating your overall symptoms aggressively before damage becomes permanent (which was Birnbaum's reasoning for the reduce positive results after the first year).

You need to assertively convey that message that you want to have your symptoms treated aggressively. You are better off finding out now whether she will or not and if not find someone who will. With PN, if the cause is treatable and before too much irreppable damage is done time is of the essence

This is why I came here to as Enbloc - your advice over the past few years has educated me enormously so I need my tutor (i.e you!) to add the finishing touches, as it were.

I ask the first question because Raynauds was my first diagnosis re the SFN burn/freeze. My fingers and toes don't get the full gamut of colours but the do go bright, luminous white occasionally - my toes do this everyday in winter months. A diagnosis of CREST would depend on a long history of classic Raynauds but a diagnosis of diffuse Scleroderma would perhaps explain a more diffuse, neuropathic type of secondary Raynauds I think? Certainly this is what my old GP thought anyway. And a rheumy professor of vasculitis supported his theory. Nifedipine did seem to help until it caused a terrible follicular rash and Sclero can also cause a diffuse SFN and facial problems too. I don't wagtail to be misdiagnosed with a presumed SFN without checking this out thoroughly.

Here in the UK, particularly Scotland, doctors are much more wary of prescribing all medications than they are in the States. So if the neuro abd rheumy decide that I just have a glandular Sjogrens with presumed SFN then I'm doubting very much that they will think this warrants Cellcept or IViG. That's just the reality of the situation for me presently I suspect

The SFN is really flaring just now and I have a numbness thing going on around my eyes, bridge of nose and mouth. It's driving me vaguely nuts! Otherwise I'm feeling quite positive and looking forward to being a challenging patient if required to be.

But I can't imagine I'll qualify for IViG without the punch biopsies and am not that confident that I'll get a positive result this time either. I really don't want to be diagnosed with Fibromyalgia, secondary to my SS - and that's why I'm giving it all so much thought now.

The period on steroids last year confirmed that much of this SFN is part of an inflammatory process. Every time I dropped a dose it flared.

Glad you seem to approve of all my questions, apart from, possibly, the Raynauds oneX

Thanks - yes I'm absolutely aware of this (numb around eyes and tinnitus are a great incentive) - hope she won't soft soap me but if not the new rheumatologist will hopefully put her right. I will phone the helpline person from the British Sjogrens Syndrome Association and get names of SS specialists if I feel this problem is not going to be properly addressed.