The Australian Sjögren's Syndrome Association
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Undiagnosed newbie - autonomic symptoms.

Hi all,

My health has been terrible for many years but I will try and be brief! I've had many tests and my definite diagnoses are Hashimotos, Ehlers Danlos Syndrome and PoTS. I've had negative anti body tests for Sjogrens several times, same with RF and ANA, however recently my ESR has been slightly raised - max 37 and my autonomic symptoms are so much worse they are considering autonomic failure and I have more tests to do for that.

Recently pain has been so much worse along with wildy fluctuating blood pressure. I have dry eyes which are worse some times than others, terrible dental decay and receeding gums (this can happen in Ehlers Danlos Syndrome also) . I haven't been able to work for the last 4 years and wondering even if previous tests have been OK if I should maybe persue seronegative Sjogrens? Thing is, most Rheummys seem to say if ANA and RF + antibodies negative then highly unlikely sjogrens. What to do?

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It's a tricky one. The thing is that official seronegative Sjögren's requires a positive lip biopsy result. And if you are seronegative, as I am, then the autonomic and other neurological systemic features are more likely to occur but less often diagnosed because Sjögren's is still thought by many to be a disease affecting the moisture in eyes and mouth. It is also very often regarded as untreatable if you are seronegative - in the UK anyway.

I plan to post an update here soon about my recent connective tissue disease appointment and oral medicine review too. I am on the maximum dose of an immunesuppressant called Mycophenolate/ Cellcept and am to be tested for gastroparesis - which is autonomic dysfunction of the digestive system. My thermostat is stuck at freeze and I can't sweat after exertion but can wake dripping suddenly in the night. My breathing is sometimes slightly erratic just now. My BP was aleays high but has come right down recently and my urine has been clear of blood for first time in years - but some days it fluctuates wildly. Some days my heart beat is too rapid, others if feels slow. I'm hypothyroid and have a very widespread small fibre neuropathy.

I was initially misdiagnosed and treated for RA but, once my ANA finally showed clear positive last year, I was given a lip biopsy that was 100% positive. My ESR is often in the 60s but we all have different normal levels. The oral consultant and my dentist were amazed at my positive lip biopsy because my mouth is not that dry and my teeth are good. My rheumy and optician and neurologist, were less amazed but still said my biopsy was unusually positive.

But if I fail Mycophenolate I'm told there is nothing more that they can offer me for this neurological type of Sjögren's. I'd still prefer to know what I'm dealing with than not but it is very depressing as there are no targeted systemic treatments for seronegative Sjögren's. I live in Scotland so am an NHS user and this is a UK report but it may help you to make decisions about whether it's worth having a lip biopsy done or not. academic.oup.com/rheumatolo...

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Thing is i do feel systemically dry, now getting alot of very painful systemic nerve pain, its almost like my nerves are inflammed. I thought I read they can try immunosuppresants or steroids? My mouth is on the dry side but really not too bad, I intermittently have issues weeing but luckily it seems to have resolved itself before I end up getting catheterised. I don't sweat at all anymore and am exercise intolerant. My diet has been changed fairly radically; low carb, no gluten dairy or soy which does help gut stuff.

I wonder if they re did my ANA/RF test they might ever be positive? I've landed on my feet finally at a hypertension clinic at St Barts, London and if there's a cause or treatment available then I'm sure it will be forthcoming, they are doing lots of tests. Previously I was being batted around local hospitals getting absolutely nowhere. I can't imagine there's a huge amount of expertise in some parts of Scotland, that's certainly the case for my other conditions. Poor you. I will say though it took 22 years to get the PoTS and EDS diagnosed but again have a really good cardiologist at Kings College London now.

How do I convince them a lip biopsy is a good idea and are there any good Sjogrens rheummys in London?

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The top Sjögren's rheumy is in Swindon. She is meant to be lovely and is called Dr Elizabeth Price. She does take private patients one morning a week. You can only be officially diagnosed with Sjögren's by a rheumatolgist and they would refer you for a lip biopsy. If your bloods are still negative then you may have the version I have which is very like MS. This link might help: hopkinssjogrens.org/disease...

My rheumy team usually see Scleroderma, Vasculitis and Lupus patients and the RA clinic sees the Sjögren's patients. But I've been very lucky to get into this CTD clinic and generally, since moving to the Dundee area my doctors all seem very gemmed up on Sjögren's. My first rheumatolgist was in Aberdeen and was nice but he was very much an RA man. Second one was a pedantic little man who told me that I didn't have a connective tissue disease - I was still on steroids when he tested all my antibodies. Then we moved to the Dundee area last year and bingo!

I am on an immunesuppressant for mine and have tried 4 others over a period of six years - but I think I'm quite unusual in this respect. Many only get given Hydroxichloraquine - which isn't an immunesupresant but is a DMARD.

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Thing is this could just be Ehlers Danlos Syndrome but I would really like Sjogrens ruled out once & for all in case it opens up any other treatment option I suppose. Thanks for sharing, are there any other UK places for support info?

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The British Sjögren's Syndrome Association is worth joining if you have Sjögren's. They have groups who meet up in some areas of UK too and a really informative magazine. I know that many sufferers have EDS as well so I would say that it is well worth pursuing a diagnosis of Sjögren's if you feel that this might help.

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Just thought I'd update you Twitchytoes. Pain was SO very bad i threw caution to the wind and ended up seeing a wonderful Rheumy who has diagnosed me as definitely Sicca, small progress. So thats 2 hurdles down; dry eyes and mouth and she's referred me for a salivary gland ultrasound which is booked for tomorrow - have pushed the boat out to get things moving fastish and gone private for those 2 steps. If I do need a lip biopsy I can get referred to her on the NHS and save a packet but by that time it will be almost certain either way with any luck...

Seems I made a brilliant decision on my choice of doc, she's already written a paper you may know of and if you don't you should: internationaljournalofcardi...

My hypertension doc also happens to be included as a writer of that paper so I seem to be in good hands. Please wish me luck for the U/s tomorrow, am scared witless as this could be the answer I have been searching for for 20+ years. Yikes!

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So sorry Jaydee I should have followed your post here but never received a reply alert!

How did your apt go? I admit I'm really baffled that you've sent me a cardiology paper because Sjögren's isn't particularly associated with cardiology issues apart from PoTS? But anyway I can't open it to read it in full I'm afraid.

However only a rheumatolgist can formally diagnose Sjögren's and Swindon really isn't far from you if you're in the London area anyway.

So - whether or not your parotid ultrasound was normal - as mine was same time as my lip biopsy came back highly positive -I do really recommend that you book an appointment to see Dr Elizabeth Price. She is the only rheumatolgist in UK who dedicates her working life to Sjögren's and what she doesn't know about this disease - no doctor in UK does.

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I saw a Dr Sutcliffe who runs a sjogrens clinic and also does research, she's very interested and has written a number of papers. It's the autonomic neuropathy side of things I think that's sjogs? The guy in the paper turned out to have seronegative sjogrens, no I can't open the paper either but you can read the gist of it

Anyway, the ultrasound was negative, the doc said negative for sjogs so I was pretty deflated, although now you've said yours was negative too but lip biopsy was highly positive I'm confused. lol! The doc today said the unltrasound was almost 100% but that's not what the rheumy said - more like 82% So now I'm not sure what to do. Maybe go back see the rheumy and discuss further? I'll call her secretary next week and discuss it. She said I had plenty of things indicating sjogrens but maybe nothing but lip biopsy going to show up? Maybe it really just is the Ehlers Danlos drying me out?

Tell me what else you know about the reliability of the ultrasound please?

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Hi Jaydee1507

I am in a similar situation as you were above...how have things progressed for you re the lip biopsy? Did u ever get one? I hope things have improved for you.

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Oh Hello. :) Am now waiting on lip biopsy appointment. Meantime I've seen a Nutritionist who thinks I have leaky gut and have started on L-glutamine. Been on that 3 weeks now and it's making a difference but time will tell.

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Hi Jaydee1507

How are you going? Did you receive results of your lip biopsy yet? I hope you are being managed as best as possible with all your symptoms.

Kind regards

Jar1973

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I'm struggling along thanks. Have lip biopsy booked for August and see dental & mouth people before then. Thanks for asking. :)

Also, what about you? How are you doing?

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