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Probably Sjögren's Syndrome all along.....

Discussion in 'Connective Tissue Disorders/Ehlers-Danlos Syndrome' started by Megan17, Mar 29, 2016.

  1. Megan17

    Megan17

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    Hello all,

    I have returned to Phoenix Rising after a five year absence only to find that there are no specified autoimmune forums anymore...but no matter.....so hopefully the Connective Tissue forum will suffice.

    Since 2009 I have had the 'Fibromyalgia' label (given by a Rheumatologist), which I have always held at arms length, not being thoroughly convinced.

    In 2007 I became very ill with many symptoms: extreme fatigue, neuropathy, muscle and joint pain, heavy eyelids, loss of balance, nausea, weakness with swallowing, fasciculations, geographic tongue and a few others. I saw two neurologists both of whom did many tests ranging from brain MRI, NCS (nerve conduction studies), Coeliac AB's, SPEP, GTT, LFT's, ANCA, ANA, ENA's to name a few. There were no significant abnormalities. In particular the ANA's and ENA's were negative. These were repeated about two years into my illness and again were negative.

    Between then and now some symptoms have lessened like the fasciculations and the extreme fatigue. However other symptoms have emerged like orthostatic tachycardia, peeling skin in mouth and pain in eyes. I have also been diagnosed with Gastroparesis, with Bronchiectasis and with Peripheral Neuropathy.

    Discounting the Fibromyalgia diagnosis, I have felt that all of these separate diagnoses were not disparate events but likely linked under one umbrella diagnosis. Their timing of onset was too compressed to be coincidental.

    Recently (2016) while at my Pulmonologist's appointment he said to me "I am seeing an autoimmune picture here with these results". His statement prompted me to go to my GP and ask for various immune marker tests to be done. These were taken and a few days later my GP asked for me to come in to see her.

    She began by saying that my results were pointing to a Sjögren's Syndrome diagnosis. The ANA titre was raised to 1:1,280 and the SSA Anti Ro was positive as was the Anti Ro52. The SSB Anti La was negative. I have now been referred to a Rheumatologist for confirmation of diagnosis.

    So the old adage that it takes about six years to get diagnosed with Sjögren's, often because the autoimmune markers may not rise for several years has been true in my case.

    Paradoxically it is a relief to get 'abnormal' results after living with my own scepticism for so long.....leave alone anyone elses!



     
    Last edited: Mar 29, 2016
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  2. charles shepherd

    charles shepherd Senior Member

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    I take the view that Sjogren's Syndrome (SS) is an important part of the differential diagnosis of ME/CFS - because there are a number of overlapping clinical features in SS and ME/CFS in addition to debilitating fatigue.

    It is interesting to note that dorsal root ganglionitis (DRG) has been found in SS and that the UK post-mortem research group have found DRG in some of the post-mortems we have reported on

    Information on SS from the Clinical Assessment section of the MEA purple booklet:

    Sjögren’s syndrome (SS): A number of viruses, including Epstein-Barr virus and endogenous retroviruses, have been implicated in the causation of SS, as well as an (CS: OK) uprated cytokine profile. The commonest presenting features are dry eyes (causing a gritty sensation) and a dry mouth. Vaginal dryness (causing painful intercourse) and respiratory tract dryness (causing a dry cough) may also be present. Debilitating fatigue and/or arthralgia is present in a significant proportion of cases. Anti-Ro and/or anti-La antibodies are found in approximately 75% of SS patients. Hypergammaglobulinaemia with raised immunoglobulin G (IgG) and/or IgM is common. A Schirmer test assesses ocular dryness.
     
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  3. Jonathan Edwards

    Jonathan Edwards "Gibberish"

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    Dear @Megan17,
    Have you suffered from post-exertional malaise do you think?
     
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  4. Andielyn

    Andielyn Senior Member

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    @Megan17 Interesting and glad you have a diagnosis which seems to fit. It is interesting that I have many of the symptoms of Sjögren’s syndrome. I have high levels of EBV, have in the last few months been diagnosed with dry eyes and have other dryness symptoms. @Jonathan Edwards I have PEM, or at least from how it is described and the flares I have match it, with debilitating fatigue. Why do you ask? Is that a differential diagnosis or also a symptom of SS?

    I recently had brain CT then follow-up MRI for severe migraines which showed for "hypodense white matter lesions". I see a neurologist in June. I had a good week last week then Friday I got slammed hard with PEM...I pushed beyond my threshold.

    I understand the paradoxical relief of getting abnormal results. :hug:
     
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  5. Maria1

    Maria1 Silence speaks volumes

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    Hi @Megan17

    I wonder if having a diagnosis of Sjogren's means you can start some treatment that will help with your symptoms?

    I am currently awaiting tests for Sjogren's. I was diagnosed with CFS just over 3 years ago, then Autoimmune thyroiditis about 18 months ago.

    My ANA and other rheumatology blood tests I had a while back were negative.

    I have suffered from unbearably gritty eyes for years, pre-dating CFS/ME symptoms, use prescription eye drops. I was abnormally tired for around ten years and had the odd TATT blood test but all were reported normal.

    I have had a strange dry cough for a good few months now. A strange sensation when I breathe in, as if I can feel the air going in; my airways just feel so dry. I actually wondered if I was becoming asthmatic, although I didn't think this was possible. Chest X-ray was normal and GP brought up Sjogren's as I happened to mention cough whilst getting a new prescription for better eye drops. I've gone in circles with the GP practice for diagnosis (don't seem keen on rheumatology referrals) and went to my optician in the end who referred to eye specialist.

    I've also had periods of very dry mouth over the years, but have put this down to ME or thyroid. It's not actually so bad at the moment. My dentist has also offered to refer me to an oral surgeon although he says my mouth isn't extremely dry, it is dry enough for referral.

    It's interesting you say autoimmune markers may not rise for several years. The only reason I'm chasing a diagnosis is that my instinct is that this isn't ME, but I may just be wishful thinking. My PEM seems much less than it was in the first 2/3 years (if at all, though 'm being careful not to test it too much!)

    Like you, as awful as it must be to be diagnosed, I can understand the relief. Wishing you all the best.
     
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  6. charles shepherd

    charles shepherd Senior Member

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    As mentioned earlier, there are a number of tests that are used to make a diagnosis of Sjogren's Syndrome (SS)
    These are described in more detail below

    From what you say it sounds as though SS does need to be considered as a possible diagnosis here and I assume that you are being assessed by a rhematologist in relation to SS

    Confirming (or excluding) a diagnosis of SS is obviously important because there are a number of treatments that can help with both symptoms (dry eyes, mouth, joint pains, cold feet etc) as well as the underlying disease process

    But it would not be appropriate to start using immunosuppressive disease modifying drugs for SS unitil a diagnosis had been confirmed and it was felt that this approach was necessary

    Assessment of SS

    Dryness of the eyes and mouth can have other causes, which include:
    • sicca syndrome, which means the glands are less able to make tears and saliva – this can occur as you get older
    • conditions other than Sjögren’s syndrome, such as sarcoidosis, which can also damage your tear or saliva-producing glands
    • certain medications such as antihistamines and antidepressants
    • inflammation of the oil-producing glands in your eyelids (blepharitis), which causes excessive evaporation of the tear film
    It’s important to see your doctor to get an accurate diagnosis. Your doctor will ask about your symptoms and carry out tests to assess how dry your eyes and mouth are.

    Because there are a number of possible symptoms and complications with Sjögren’s syndrome, it's likely that you'll also need to see an eye specialist (ophthalmologist), a dentist or oral surgeon, or a rheumatologist before you’re diagnosed.

    What tests are there?

    The main tests that can help with diagnosis are:

    Tear production – A small piece of sterilised, pre-packaged blotting paper is used to measure your tear production. This is called Schirmer’s test. The paper is placed in the corner of your eye and folded over your lower eyelid. This is a little uncomfortable and causes your eyes to water, making the paper wet. The amount of water that soaks into the blotting paper within 5 minutes is measured, and this tells the doctor how good your tear production is.

    Eye examination – An eye specialist will put a dye (fluorescein and sometimes lissamine green dye) into your eyes and use an instrument called a slit lamp to examine them. The lamp shines a beam of light through a narrow slot (the slit-beam) and magnification allows the doctor to examine the surface of your eye more effectively. This makes it possible to see the film of liquid over the surface of each eye. If you don’t have enough of this liquid, it could be a sign that you have Sjögren’s syndrome.

    Saliva production – You may be asked to spit or dribble into a container over a period of 5 minutes or so. The amount of saliva that you can produce in that time is measured.

    Ultrasound scans – These are commonly performed now to help diagnose Sjögren’s syndrome. The salivary glands usually have a uniform grey appearance on ultrasound images. In Sjögren’s syndrome, round black areas can be seen in the scans.

    X-rays of your salivary glands and ducts (sialography) – A contrast dye is injected so that your glands and ducts show up clearly in the images. This is rarely performed for diagnosis now and is mainly used to identify blockages of the ducts in people who have repeated salivary gland infections.

    Other scans – Occasionally magnetic resonance imaging (MRI) scans are used if there is uncertainty over the diagnosis or if lymphoma or other abnormality is suspected.

    Blood tests – People with Sjögren’s syndrome often have high levels of antibodies in their blood. These can be measured with blood tests. High antibody levels can make your blood thicker than usual, and this is measured by an erythrocyte sedimentation rate (ESR) test. The ESR measures how fast the cells in a tube of blood settle. The thicker your blood, the faster the cells settle and the higher the ESR.

    People with Sjögren’s syndrome often have very high ESR levels regardless of whether they feel well or ill. In Sjögren’s syndrome, the ESR or other measurements of inflammation such as C-reactive protein (CRP) aren't very useful in assessing how active the condition is – unlike in lupus or rheumatoid arthritis.

    Two particularly important antibodies are called anti-Ro and anti-La antibodies. They’re found in 75% (anti-Ro) and 40–50% (anti-La) of people with primary Sjögren’s syndrome and can also be seen in patients with lupus. If you have anti-Ro and/or anti-La antibodies along with dry eyes and/or a dry mouth, it’s very likely that you have Sjögren’s syndrome.

    Lip biopsy – Several tiny salivary glands may be removed from your lower lip under a local anaesthetic and examined under a microscope. This is increasingly being done to assess the future risk of lymphoma. If there are no early signs then the possibility of developing lymphoma in the future can almost be ruled out. And if the test does suggest a possible risk of lymphoma then this can be monitored and treated as appropriate.

    Further specialised tests may be needed if you develop swelling in the lymph glands (in the neck, armpits or groin) or if you have complications involving the chest, kidney, liver or nervous system.

    - See more at: http://www.arthritisresearchuk.org/...-syndrome/diagnosis.aspx#sthash.Uwdq6Wix.dpuf
     
    Last edited: Mar 29, 2016
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  7. Andielyn

    Andielyn Senior Member

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  8. Maria1

    Maria1 Silence speaks volumes

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    Thank you @charles shepherd

    Just for information in case others are wondering about a diagnosis...

    Although I am registered with a fairly forward thinking GP practice, since the GP first mentioned Sjogren's, it has involved several return trips to get to the point I am now, and I'm still not very far along the road of excluding or getting a diagnosis. It's hard because I'm knackered and, as we all know, knocking on doctors' doors is just exhausting.

    The GP who first suspected Sjogren's said he needed to discuss with a colleague how to get hold of the blotting paper strips, and to come back to him if I didn't here within a week or two. When I finally went back weeks later having heard nothing, the GP with which he was going to consult just knocked me back to my endocrinologist, even though it was obviously not that specialism.

    It's my optician and my dentist who have been the most straight forward route for a referral.

    Although I have asked my optician at every visit about my dry eyes, they have never done anything about it except say oh yes they are quite dry. This time I made an appointment just for the dryness and they put some dye in and said severe dry eyes need referral. I'm assuming that may mean some of the tests in your post. I think the only way I'll get a rheumatology referral is if these tests indicate Sjogren's.

    @Megan17, It seems you have gone a long time, really poorly without a diagnosis. If I do get diagnosed now, I think it will be unlikely as my symptoms really aren't severe. I hope you can get some relief.
     
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  9. charles shepherd

    charles shepherd Senior Member

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    Hi Maria

    Here is some good simple info on the Schirmer's (dry eyes) test:

    http://www.healthline.com/health/schirmers-test#Overview1

    It sounds as though one of your medical team needs to get this organised

    [​IMG]
     
  10. Maria1

    Maria1 Silence speaks volumes

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    Thanks @charles shepherd

    I'm expecting the Schirmer's test is what the Eye specialist will perform following my optician's referral.

    If not I'm half considering you-tubing a DIY test!
     
  11. Andielyn

    Andielyn Senior Member

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    Again, thanks for the education. I went to my opthalmologist for eye pain and sudden onset of blurry vision and was diagnosed with dry eye syndrome but without this test. I have been using drops and a personal humidifier. I also have hyertopia, which is new and a rapid onset. I suspected both were caused by Topomax for migraine prevention but stopping the med has not resolved my dry eyes or blurry vision.

    @Maria1 I hope you get some answers. Best of luck to you.
     
  12. Megan17

    Megan17

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    For the first five years I believe I suffered from PEM. It was quite scary (not telling you anything no doubt ) when one couldn't get out of bed for eg. two, three or more days, I used to think I hope this doesn't last forever .....I want to get back into living....such as it was, I have so much I want to do (don't we all?).

    Initially I spent the first four months more or less unable to get out of bed which was less to do with PEM but rather the acute phase of whatever I was experiencing. However once I was able to start to do some more things, albeit limited, if I overdid it I definitely experienced a crash usually the next day but sometimes within hours.

    It has been awhile since this last happened.......thankfully.

    Was there a reason you asked Jonathan?
     
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  13. Maria1

    Maria1 Silence speaks volumes

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    @Andielyn

    Thank you- I hope you get some answers too. I'm also on topirimate for migraine. My dry eyes pre- date my starting that however. I stopped it for a while but am back on it now as the migraines returned with a vengeance.

    The blurred vision rapid onset you mention is also interesting- again I put this symptom down to ME and/or thyroid. My optician just keeps giving me new glasses!

    I have noticed there are a few people with Sjogren's on here who keep the ME diagnosis. I'm assuming PEM is a key factor in this.

    Glancing at thyroid forums, people talk about the need for pacing and 'crashing' is talked of a lot. My endocrinologist asked me if I 'pay for it' when I get a burst of extra energy after a dose increase of thyroid meds, so he is obviously aware of this crashing syndrome too.

    It would be interesting to study the difference between auto immune crashes and the PEM of ME. I know my PEM was specifically delayed onset and brought about by the slightest exertion in the early days of my illness. Now my 'crash' will be the next day, if at all, and then I can push myself further.
     
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  14. Megan17

    Megan17

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    Hello Maria,

    Thank you for your response.

    It would certainly be amazing if I could start some treatment to help this condition (if indeed it is Sjögren's). My appointment for the Rheumatologist is 19th May but I am on a cancellation list should one come up before then.

    A question I'd like to ask people is regarding what meds/treatment SS people have found to be most effective for them and how.....? I know there is Methotrexate, Plaquenil, possible IVIG, and Ritiximab. I may post this as a separate question for feedback.

    I think the term 'dry eye' is not a very useful term to use when trying to elicit whether a person has it or not? Some medical and SS sites use it in questionnaires. When I was asked originally by a doctor if I had dry eyes I said, "No, I don't think so". Duh! A better question would have been, "Do you have any eye symptoms?" Yes I certainly had several eye symptoms like.......pain deep in the eyeball, constant tearing when outside in the sun (didn't sound like dry eye to me!) heavy eyelids (difficulty opening them), blurred vision and painful cracks at the outer corners of my eye.

    After responding to my ME/CFS/Fibromyalgia doctor in 2012 that no I didn't have 'dry eye' (hehe) he referred me to an opthamologist who did a Schirmers test. The result was that I had 1mm of tears from my left eye and 2mms from my right eye. This fell well within the category of 'dry eye' and the opthalmologist in his report felt that I possibly had Sjögren's Syndrome.

    Unfortunately that's where the diagnostics all stopped and I'm now kicking myself that I wasn't more proactive in following this up with antibody testing. I'm not a very good advocate for myself sometimes although I was in the early days of this illness. However I think I got so much negative innuendo that I got beaten down eventually and lost confidence. My GP was hopeless and the M.E. doctor didn't raise having repeat ANA and antibody testing done. I asked him if I should see a Rheumatologist and he said probably not for the moment. I have no clue why not. Anyway I guess it is water under the bridge now so I need to move forward.

    Don't be like me Maria. If you believe that there are strong indicators pointing to a particular diagnosis, keep pushing. As I said, it is well known that sometimes antibodies do not rise for years after the onset of an autoimmune condition.

    I hope you get to the bottom of whether you have Sjögren's or something else.

    Talk again soon, no doubt!
     
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  15. Jonathan Edwards

    Jonathan Edwards "Gibberish"

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    I do not pretend to know the mechanisms of ME/CFS any more than anyone else but my suspicion is that there may be a range of causes, some of which may be autoimmune and some 'autoinflammatory' like Reiter's syndrome. As a rheumatologist the people I saw with severe exhaustion tended to have Sjogren's or other ANA-related conditions or Reiter's. I never specifically explored the concept of PEM with them but it was my impression that they had 'crashes' and delayed problems after exertion. So I am tempted to think that ME/CFS may be a common pathway that can arise from Sjogren's, Reiter's, EBV or whatever.

    We have had recent discussions about how specific PEM is and I guess my thought is that if the 'other sorts of fatigue' are things like Sjogren's than maybe it is not a discriminator between the two. If you have high titre ANA and anti-Ra (La is less important) then it seems pretty certain that you do have primary Sjogren's. So your history is an acid test of the place of PEM I guess.

    With regard to treatment: Treatment for the things that matter in Sjogren's has been disappointing in the past. I am not sure that methotrexate would do much - it was not routinely used. We had hoped that rituximab would work as it does for RA and probably lupus but anti-Ro antibodies do not go away easily with rituximab. There have been some trials and they have been positive in some respects but I am not clear how useful it is. So missing out on Sjogren's treatment in the past is probably no great loss - you may have avoided side effects and little else. Hopefull within the next few years more powerful B cell targeting drugs will come along so that the more resistant autoimmune conditions may respond as well as RA. Rituximab may be worth a try but I have not seen recent discussions on this.
     
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  16. Megan17

    Megan17

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    Jonathan's question about P.E.M. brought me back to a topic which I have not thought about for four or five years.

    Approx five years ago my M.E./CFS doctor started me on low dose Propranolol which is a beta-blocker. He prescribed it for orthostatic intolerance (OI), more particularly for orthostatic tachycardia. It had become a troublesome (autonomic) symptom for me with my heart rate increasing to between 120-150bpm after standing upright. Almost immediately I noticed an incredible difference in that my heart rate started to behave just like it should......

    .......furthermore the correlation between starting the beta-blocker, diminuition of orthostatic tachycardia and the virtual eradication of P.E.M. was dramatic. In fact from memory, I would venture to say (without the benefit of a diary record) that my episodes of P.E.M. significantly stopped at that point.
     
    Last edited: Mar 31, 2016
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  17. Marco

    Marco Grrrrrrr!

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    @Megan17

    As you now appear to be the forum guinea pig for things Sjogren's related have you noticed any changes in the pattern of temperature regulation or sweating (either reduced or greatly increased)?

    That seems to be a common though little discussed problem with Sjogren's which some put down to the side effects of meds while others believe in is one manifestation of autonomic neuropathy.
     
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  18. jimells

    jimells Senior Member

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    Uggh. There is no way I could calmly sit there with paper stuck in my eyes. How can that person tolerate it?
     
  19. Sidereal

    Sidereal Senior Member

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    I've had this done twice and I have to say it seems far worse than it actually is. And I'm the world's biggest wimp when it comes to invasive medical tests.
     
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  20. Amaya2014

    Amaya2014 Senior Member

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    I'm wondering how people interpret crashing. For me I hit this "wall" where my body becomes like lead and I'm struggling to walk, hold my head up, think, even simply breathing. All the other symptoms like pain, sensitivity to light and sound and temperature and smells ramp up to intolerable, and I literally become like a useless piece of clay or a rock. At these times even simple things like writing my name or performing a math problem become Herculean tasks. Emotionally I'm labile, angry and frustrated at my helplessness, strongly desiring to execute whatever plans I'd made or complete the task I was trying to do, and tearful at the hopelessness of my situation. And then I'm forced to shut down as there is no other option until I regain some degree of function. Is this how others are experiencing "the crash"?
     
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