Ehlers danlos, Sjogrens and A.S

Hi everyone, I'm on numerous forums but this was suggested to me as a good place to come!

I'm 38 and have been very unwell for about 4 years. Only this last 6 months after seeing endless consultants did I learn I have EDS hypermobility type and sero negative Sjogrens. Had A.S for a long time. I suffer terrible neuropathies from the sjogrens including full body small fibre neuropathy, autonomic neuropathy and more recently trigeminal neuralgias which have affected my swallow, my ability to chew, feel etc. It has been quite an awful journey and I have had to fight to get my diagnosis as no one would entertain possibility of sjogrens without positive bloods. It was only through the support of forums like these that I went and had the lip biopsy privately in the end. I was almost convinced out of my suspicions that I had sjogrens by most rheumatologists I encountered. In the end I listened to the forums and ignored the rheums, they were wrong in their insistence I did not have sjogrens.

I'm currently in the system trying to access co ordinated and well informed treatment. This has not been easy at all as there is no clear direction in this country on this it seems. I am on plaquenil which I doubt helps the neuropathies at all but I will persist.

I really look forward to getting involved in this group despite the fact my diagnosis is Sjogrens and not Lupus. Thanks for having me! 😁


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25 Replies

  • Hello Joanna.I'm sorry you have had such a difficult time getting a diagnosis.

    Have you managed to find a decent Rheumatologist who knows more about S S ? I don't know where you live but if you are in the UK I would thoroughly recommend Dr Elizabeth Price in Swindon. Others will know of others.

    We have someone else on this forum with almost identical problems with the neuropathy side of things which are really badly affecting her life. Keep an eye out for her posts. Her name is ' Twitchytoes ' and she has done a lot of research on all of this.

    My SS has had to take a back seat for a while. I hope this has been at least a little helpful Good Luck.

  • Hello Joanna, Welcome! I am also not diagnosed with lupus but it is such a friendly and informative board that I lurk about still! I was diagnosed with AS in October after 26 years of symptoms that were often stopping me from functioning. I was continually told it was all in my head and only got my very first referral to a specialist (in any discipline!) a year ago after testing positive for HLA-B27. Even then the GP was very reluctant to refer but thankfully she did, as the MRI the rheumy ordered showed masses of inflammation. I've not fused yet but I have practised yoga for many years and I think that has helped.

    I also suspect I could have other issues going on - my tear evaporation rate is 4, my mouth and lips are very dry and the rheumy commented that I am overly flexible in my hips, elbows and knees. Currently I am coping with Naproxen although my rheumy immediately started talking biologics but I wanted to go away and have a little read up about all these drugs before making my decision. Today is a bad day however and I am sitting here with my heat packs trying to get some relief and do some work! Biologics look tempting today! One of my biggest problems is that I don't really know anymore when I am in pain, I presume as it has become normal for me and I can tolerate a lot now. I kept telling the rheumy my pain was about a 3 and not a problem, it was fatigue that was causing me most distress. I did clarify by also telling her that I found childbirth totally bearable as well and didn't need pain relief then either but it wasn't until she saw the MRI that she accepted that I definitely do have a high tolerance to pain! I actually refused the naproxen for months until she said she needed me to take it as it could also be a diagnostic tool - felt a right banana when I did take it and it made a massive difference :)

    Have you joined NASS? Their forum is not well used, but they do have lots of very helpful information leaflets and a helpline. They also run groups in many areas where you can go for hydrotherapy and meet others with AS.

  • A very warm welcome here. As you have found already we are a friendly helpful bunch with lots to share and shedloads of support 🌈

  • Thanks jnmmum! Yes i was part of NAS and hydro groipnforba few years. I've become so unwell with neuro problems that I can no longer go. For me the sjogrens is the big baddie causing my most awful problems. I was offered enbrel, took it twice and nerve pain went through the roof next day. It's a difficult predicament as there are issues with this when you have neuropathy. Then another guy I saw said he would not use in my case with what I have from sjogrens. If I didn't have nerve damage I would use it by the way.

    So I'm begging for one person to take a view of the entire situation and advise on what next. The disjointed approach to care makes this so very difficult

  • Yes I know! Getting anyone to look at "all of me" has been nigh on impossible! Even when I was positive for HLA b27 the GP told me I could be positive and not have any problems ever. After 10 minutes of this, my husband (who thank goodness was with me) interrupted and said "But she DOES have symptoms - that's why you tested her, so you need to refer!" At first the rheumy wasn't really buying it, but I persevered because she was the first specialist I had ever seen, but to give her her due, she did thoroughly test and discovered the masses of inflammation. Next appointment I need to start asking about all the other stuff and getting to the bottom of that. Do you have GI issues? I was told I had IBS, yet the omeprazole I take with the naproxen completely stopped all symptoms overnight... There is something else definitely going on there and my next aim is to push for further investigation on that. As for neuropathy, I get numbness intermittently in my upper arms, hands, at night - it wakes me up. I'm not laying on them, they just get weirdly numb yet pins and needly iyswim. Then weeks of nothing...

    Another issue is getting people to say AS correctly and understand what it is doing to me. I'd never even heard of it last year, so it's all new to me too. My husband is very much TAKE THE BIOLOGICS and I am patiently trying to explain, there are side effects, you have to weigh up if they are better than the disease progressing, they can make no difference, make things worse, improve things - there seems to be no rhyme or reason to how people react. It's my body and my disease and I just have to deal with it in the way that works best for me. I've not said no to biologics, I just want to be better informed before I make the decision :) I also want to know if there are other auto immune diseases going on as this will, as you mentioned earlier, have a bearing on what meds are safe to take! I'm not a guinea pig :)

  • I have same sounding pins and needles from my neck down both arms into my finger tips. I’m waiting to hear back from my neurologist about whether this is part of the all over small fibre neuropathy or whether it’s neck related.

  • I wondered if the bulging disc the rheumy told me I had was in the neck and was pressing on a nerve resulting in this sensation, but I'm still waiting for the copy of the letter from her to my GP so don't know. It is weird and actually painful at times - and you know once you wake up at 4AM you are not going back to sleep that night :)

  • That does sound quite likely for you doesn’t it? I asked GP abd neurologist about this possibility - although I don’t often have pain or discomfort in my neck that you describe.

    The exception is when I’m a passenger in the car and need one of those curving memory foam flight cushions now even for short distances. Like my ankles my neck often feels too weak?

    I did have an MRI done of my neck along with EMG and Lumbar Puncture 3 years ago. All that showed up was wear and tear arthritis of my neck with early C6 root changes. My GP hopes that neurologist will order a follow up MRI now. But given that I have small fibre neuropathy everywhere it’s possible that she won’t bother.

    My pins and needles are completely bilateral and also affect my lips and the rest of me is numb so I’m guessing that nerve entrapment is an unlikely cause.

  • My 10 yr old daughter bless her has found out about those pillows and had been sounding me out about them. I reckon I'm getting one for Christmas!!

  • Are you on the sjorgens site? It is really useful for information , support etc. I have sjorgens . Have you been given anything medications to help ease your neuropathy? I’ve been through trigeminal neuropathy, several times over comes and goes depending on what trauma I am going through facially. The worst was when I had casts removed from my inside lips. Carbazamine helped at night with sleep, but there are a number of options ...I now take Nortriptline at night to help with nerve pains / sleep etc.

    Sounds like you’ve had an awful 4 years , hope you get good support soon.

  • Hi 25clai, yes we have tried a few things before we knew it was sjogrens. I am keen for someone to take a view on it so and lead on deciding what to try next. It's so difficult when you are rushed in and out of these appointments half the term and have seen lots of people.

    I'm on plaquenil but want guidance from those with good experience treating this type of sjogrens on which options would be beat for disease modification. There is no clear direction on this with sero neg neuromanifestations in this country

  • Hi Joanna. Reading your post was so helpful to me. I too am 38 with 4 years of symptoms behind me. At my recent appointment with my rheumatologist I begged for a lip biopsy or for her to at least explore Sjogrens but she declined and discharged me. I have all the symptoms and have failed the schirmer test twice. I'm now in a position where I'm wondering wether to go private or not but am worried about the cost. Can I ask, are you in the U.K. And did yours cost the earth? The appointment isn't too bad but am worried that the price of a lip biopsy (if they agree to do one) will be more than I can afford.

    I hope you find someone that can look after you now. As someone mentioned earlier, twitchytoes posts are very informative and helpful 🌻

  • Shellessx, I actually feel I know more about sero neg sjogrens than most rheumatologists after my terrible experience. It is wholly unacceptable that you have to fight for this but you obviously will. I don't know where you live but I saw a sjogrens specialist called Dr Elizabeth Price privately and she very kindly put me onto her nhs list for the biopsy. She had only just set up the combined eye/ rheum clinic at this time so it was easy to get me on the list. There is a huge issue with people getting diagnosed I have noticed. People can't seem to get access to the lip biopsy easily. I am speaking to many in this position and I'm miffed by the absolute lack of knowledge amongst the majority of rheumatologists. If your blood is negative their brains cannot compute for some reason.

    You could try argue with your rheum that 30% approx are sero neg and only lip biopsy will confirm.

    I am very keen for the true extent of this condition,particularly seroneg which I believe is often missed, to be accurately reflected.

    Unfortunately, once you overcome one battle to get diagnosed you have the next one to access appropriate treatment. The true scale of the problem has to be identified though and when you know what you are dealing with it can focus your mind on treatment you may wish to try pursue. There's another lady called Dr Shand at Ipswich who was happy to carry out lip biopsy for a friend of mine based on her symptoms only. We both agree that it was minor and hardly felt it in the hands of these doctors.

  • Ah that's where I've just been discharged from, although I live nearer to Colchester hospital. I was under Dr. Rita, that's what they called her anyway as her last name was difficult to pronounce.

    The other reason I was reluctant to go private (at the oaks) was I wasn't sure if I should go to someone more specialist in Sjogrens. The dr that has been offered to me is specialist in other areas of rheumatology. Where did you see Dr price? I will look her up shortly.

    Thank you so much for your reply, it really has helped me loads and has giving me lots to read about tonight.

  • Thanks Bones! I did see Dr Price but accessing the most appropriate treatment is issue due to my seroneg status. Yes I saw a few posts wherein others had the same issues as me. Thanks for your message 😁

  • Shell, Dr Price is in Swindon. I drove almost 2 hours one way just to get the biopsy. With enough of us diagnosed I wouod love to hope we may have strength in numbers and can try change things. The current set up isn't acceptable at all

  • It's not good enough at all and so many people facing the same ongoing issues! I've looked her up and now making the call. I'm happy to do the 3hr drive if that's what it takes. Thanks again Joanna.

  • Joanna36,

    Welcome. I'm sorry you are suffering with sjogrens.

    I agree with everything you say. Sjogrens is difficult to manage. Though, the worst of it, is the utter lack of awareness, coordinated care, and treatment plan.

    At first, I found the lack of support etc, especially during flare-ups, was overwhelming.

    Fortunately, I joined BSSA and HU, and find my support here.

    Take care, we are all in it together. We care to share, and share to care. X

  • Thank you webar. Yes the BSSA have been very helpful in explaining the situation to me regarding trials. Sadly they are funded by pharma so they dictate the perimeters.and sero pos will always be easier to monitor efficacy.

    I just urge anyone who suspects sjogrens and is refused biopsy to go pursue it like I did. I'm so glad I listened because I know now and am trying to spread the word. The more of us on the sjogrens registry the better in terms of progress I hope. The pharma companies want to go where there is greatest demand and the demand is there but under acknowledged.

    Yes definitely we are all in it together. I care about the sero pos as much as sero neg and don't want it to be a divide. I Really just wish for equality for the sero neg sufferers.

  • Hi Jo. We know each other through other forums of course so just wanted to give you a big 👋

    I think the reason lip biopsies are becoming such a problem for rheumatologists is because they are usually done by dental surgeons as most Rheumatology units don’t have the facilities or skills on their premises.

    I had mine done last year by the local dental hospital which is part of the university medical school. I’m not sure if they would have referred me had my ANA not been very positive but I was reluctantly offered one while living in my old island home a few years ago. But the max fax who agreed to it was so discouraging about how unuseful a diagnosis of seronegative Sjögren’s would be that I lost my nerve. Prior to that my neurologist at the time told me that Sjögren’s is one of the rheumatic diseases which invariably shows up in antibodies. Goes to show🙄

    And loss of nerves is what eventually took a more enlightened rheumatologist to refer me to the dental hospital. And this time, with +ANA and IgGs and desperation for a diagnosis on my side I agreed.

    I went to a BSSA group meeting in Edinburgh last weekend. The guest speaker, Dr Gupta from Glasgow, told us she simply can’t get dentists or other surgeons such as ENTs to agree to do lip biopsies at all. 50% of her referrals are now being bounced back.

    I wonder if this is because of the costs and time and the undeniable fact that even when results are positive, patients aren’t able to access any further treatments beyond topical ones? Nor are we able to take part in any clinical trials.

    I met someone at that meeting who has struggled with neuro symptoms for 20 years, went all the way down to Swindon for lip biopsy I believe, and even though it was positive the Edinburgh neurologist is telling her now that her neuro symptoms are due to Functional Neurological Disorder. This beggars belief really but sadly it’s what she told me.

    According to this link the risk of CNS involvement in Sjögren’s is the same as it is in Lupus so I think we are probably in the best place here and this community and charity are both wonderful.

  • Hi Twitchy! This is so depressing. The extent of the problem has to be acknowledged. Another avenue I pursued was to see am opthamologist on the high street toncheckntear film instability. This showed a big issue when the rheumatologists schirmers eye test said I couldn't have sjogrens. So they were wrong again.

    I was told by a specialist in sjogrens that I would need a nerve conduction test to prove I had small fibre neuropathy. I have it in every part of me and only a skin biopsy proved it, nerve conduction tests only measure large fibre nerves not the small unmyelinayed ones in the skin that make you fewl you are a walking fireball. With this lack of understanding it is such a fight for us. I realised there was no point continuing the conversation once this was said.

    It doesn't take much to read a few medical articles online about all this. It isn't acceptable.

  • I know Jo. I think that the problem lies with Rheumatology clinics the world over relying on outmoded methods such as Schirmers to diagnose us. My opticians have all oohed and ahhed at my dry eyes over the years - having been using dry eye products since I was a child! Same now goes for dry mouth which isn’t dry at all although throat certainly is horribly so - also nasal cavities and ears. I produce plenty of saliva these days as I’m a nose breather.

    Why don’t they ask us this and check our nostrils for dryness?! Also it’s as much about quality as quantity. My spit is horrid, tastes salty and my mouth is always rancid tasting. My tears evaporate too quickly so tear break up is almost non existent and I have severe mybomean gland dysfunction ie 3 types of eye disease. I keep on top of all this pretty well - apart from throat dryness which is terrible at this time of year due to central heating.

    It’s also possible that the reason you aren’t eligible for trials is because your Sjögren’s is classed as secondary to your AS? I think I will discuss this with my rheum when I see them as mine was classed previously as secondary to RA but has now been rediagnosed as my primary disease ie RA was misdiagnosed. They will only include pSS patients in trials for obvious reasons. This could be another reason why Ro positivity is demanded I guess?

  • Excuse typos. That should read ' to check tear film instability'

  • That's a good point Twitchy. I am no expert but I feel that the primary / secondary diagnosis is of no relevance surely? The problem is the same!

    I tried enbrel and it set my already fried nerves on fire. It's scary for me to carry on with this having had this response. It could have been a coincidence of course no one treating has a clue. They shrug shoulders and look at you like an over anxious hypochondriac.

  • Anti TNFs are famously problematic for those with nervous system disorders so you are probably right. Although my SFN pain returned with a vengeance for the first six months of Mycophenolate I admit.

    The primary v secondary thing is interesting. Yes certainly Birnbaum et al have now discredited it as nonsense but Drs Price and Gupta both maintained that primary is a slightly different, more systemic disease. I think they mean by this that primary is usually Ro positive? - which is not in keeping with what international Sjögren’s experts say at all.

    But you can perhaps see how clinical trials would use people who have Sjögren’s as their ONLY rheumatic disease - because, if there is another, then it’s hard to know what is causing what. I really can understand this because you yourself don’t always know what is due to AS or Sjögren’s so how can they be expected to?

    But as they are saying that Sjögren’s is my only rheumatic disease then I think that lip biopsy people like me should certainly be eligible!

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