My Latest Label: Sjogren’s Syndrome

me outside my immunologist's office
me outside my immunologist’s office

I am incredibly tired. I drug myself to the dentist half an hour away only to find out I was a day early! It was no one’s fault but my own, however it is still frustrating to have used energy I don’t have on getting there. No way could I do it all over again tomorrow; I rescheduled for next week. So much for 2 weeks without any sort of appointment. Oh well!

The reason I am writing despite my fatigue is because I know many of you lovely people are eager to hear about my latest visit with the immunologist, especially after my last post on how the steroid trial went. The gist of my latest health finding is I have Sjogren’ Syndrome. In some people with Sjogren’s, the body limits itself to attacking the lacrimal and salivary glands, therefore the only issues are dry eye and mouth. In others such as myself, the body is much more ambitious. The white blood cells destroy tissue in other parts of the body as well such as the nervous system, GI system, spleen, liver, pancreas, joints, and more, making it a systemic disease. I had to put a lot of clues together (MRI findings, ophthalmological findings, endoscopy findings, biopsy findings, dental history, diagnosis of POTS, symptoms) to figure out Sjogren’s syndrome because, like 30% of people with Sjogren’s, I do not have positive Sjogren’s antibodies. All of the research and essay writing was worth it. My doctor agreed it fits but is likely secondary to another disease.ย  Sjogrens is attacking my nervous system and, possibly, so is this other disease. What this disease may be (more than likely autoimmune and “Lupus-like”) is a mystery. So, yeah… I still have a mystery disease.

The treatment path is a bit up in the air at the moment, but I should know more after going to my 8th new doctor (not to mention my 4 regular doctors I’ve seen) this year. Given the fact my condition is autoimmune and we are hoping to try unconventional therapy, she thought it would be worthwhile to check in with a rheumatologist since autoimmune disease typically fall under their specialty.

For now, I am going to start taking Plaquenil. Plaquenil is considered a disease-modifying anti-rheumatic drug (DMARD). It is mildly immunosuppresive (good since my immunoglobulins are the low end of normal; we don’t want to suppress them too much) and anti-inflammatory. It may prevent further damage to my body as well as possibly reduce aches, fevers, and fatigue. There are no guarantees and it will take a few months to know if it is helpful for me or if we should try something else. I am waiting until next Wednesday to start the drug. I don’t want to start the new medication before my wedding dress shopping day next Tuesday and possibly have side effects exasperating my condition cause me to cancel.

The unconventional therapy we are hoping to convince my insurance to agree to pay for is called intravenous immunoglobulin or IVIG. IVIG has been found to be helpful for both Sjogren’s and for Dysautonomia in some cases, but according to my information, it costs 10 to 15 thousand dollars per treatment. ๐Ÿ™‚ You can see why the insurance is a bit wary of paying for its use off label. The good news is: I am a persistent researcher and I plan to put together a paper for them on all of the case study of patients with Dysautonomia, Sjogrens, or a combination or the two who have regained their quality of life thanks to IVIG. Even if I qualify for IVIG, there is no guarantee and I will still require a drug such as Plaquenil.

I am very grateful for good insurance, my education which taught me how to research, and for having found excellent doctors who are willing to listen, think, and act. It feel as though for quite awhile, I was chasing all of my various symptoms through a maze and now I have finally ran them out of the maze and am backing them into a corner with my guns drawn… and maybe a few grenades at the ready in my belt. They are quivering in their boots at each research article I read, each connection between research and my body I make, and each capable doctor I visit. They know their time is up and they will not be stealing my life away from me much longer!

49 thoughts on “My Latest Label: Sjogren’s Syndrome

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  1. Hi Jackie. I had wondered about you and auto-immune disease. I have dermatomyositis where my muscles attacks themselves and my brother has Sjogrens. Due to the severity of my disease and needing to parent two active children, I have one down the hard core medical route and have been on prednisone and had 5 years of IVIG infusions until it was deemed that they had stopped working and I had infusions of cyclaphosphamide and intravenous pred at Christmas time. These have worked a treat. My lung volumes have increased from 43% to 60%. I strongly recommend a rheumatologist connected with a research hospital. You really need highly specialised medical care. Doctors who are attending the latest conferences and aware of the latest treatments because some exciting things are happening. I am keeping my eye on rituxan.
    I just noticed that I am signed into WordPress under my other blog but its Rowena from Beyond the Flow. Good luck with it all xx Rowena

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    1. Thank you for the tips! I will definitely keep them in mind as I navigate this new area of medicine. Rituxan does look very promising for all sorts of diseases; I also have my eye on it. I’m sorry to hear how severe your disease has gotten but am happy they have figured out a way to increase your lung volumes and get your more active time with the kids!

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  2. It’s great to hear that you’re starting to get answers (kind of), but my heart broke when I read about your journey to the dentist. Anyway, I love my rheumatologist, so I hope you like yours too! Sending positive thoughts your way (especially so you’re rested for all the further research/essay writing). โค

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    1. I smiled at you understanding how horrible it is to have wasted energy on something for no reason. The receptionist was out when I got into the office, but staff acknowledged my presence, so I sat there for 20 minutes before the receptionist came back and told me my appointment was a different day.

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  3. I take Plaquenil in combination with Methotrexate and sulfasalazine for my PMR. I hope Plaquenil will give you some relief. It took several months before I saw any real difference., but on the plus-side not much in the way of side-effects.

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  4. That last paragraph sounds like our old Jackie that we all know and love. Not that you ever let it get you down too much or for too long, but I think this disease has met its match!!! I will be glad to stand by and load your guns and carry your grenades for you. You go girl!!!

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  5. Very glad you are getting answers! I have actually seen in recent peer-reviewed literature that up to 50% of Sjogrens patients are seronegative. Did you get someone to do a salivary gland biopsy? I asked my rheumatologist to order one for me, but she would not. She is unaware of the connection between dysautonomia and Sjogren’s. My plan is to go to an autonomic neurologist and be evaluated for the various types of neuropathy. If I can show the rheumatologist that I have neuropathy, then maybe she (or another rheumatologist) will order the biopsy–since Sjogren’s is well known for causing neuropathies.

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    1. I had a lacrimal gland biopsy. I really wouldn’t recommend it as the lip biopsy is much less invasive. Since I’d already had it done, it was substituted in for a lip biopsy. Hopefully the Rheumatologist agrees with the other doctor and literature I have and doesn’t request I have a lip biopsy because I do not recover well from procedures.

      I saw the study where 50% of patients were seronegative too! I included that in my essay I wrote for the doctor explaining why I think I have Sjogren’s. If you can, I would recommend doing the same thing and write a report. Read journal articles and information from The Sjogren’s Foundation on manifestations and make your case for why you suspect Sjogren’s, support your case with your established medical history, and demand a biopsy. If your ANA is elevated, I think most doctors would agree to order it, but I could be mistaken.

      Good luck with the autonomic neurologist! Maybe s/he could even order the biopsy for you. After all, Sjogren’s attacks the nervous system so it isn’t too far fetched for an autonomic neurologist to order the biopsy then refer you if it comes back positive.

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      1. I hope you can get out of the lip biopsy too! You have been through enough with that lacrimal biopsy. Yowsers!

        Yes, the autonomic specialist I have my eye on is young enough that I hope he has either read the same articles as you and I have, or will be open to reading them. Many of the doctors near me are ready to retire, and have not cracked a journal in decades it seems:-(

        My ANA is not elevated, but the rheumy who refused me the lip biopsy has seen my manifestations with her own eyes, including the fact that I have had an autoimmune pleural effusion and pericardial effusion in the past, and even diagnosed me with SICCA Syndrome. She keeps saying that it is the anti-inflammatory diet I follow that is keeping me seronegative, yet acts like she will not order a biopsy unless it shows in my labs.

        When you finish your manifesto, I would love a copy. I have lots of articles printed out, but a 4-year-old who does not like for me to do anything but dote on her for too very long, haha;-)

        If you need full text of an article, I may be able to help. I still have access to lots of journals via my former employment.

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  6. I have read many good things about IVIG and it’s positive effect on POTS and Lyme. Adding Sjrogen’s to the benefit list does not surprise me. I think it is a matter of boosting our immune systems as much as possible. Sending positive thoughts that you can get this treatment approved quickly!

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  7. You are such an inspiration!
    When the experts don’t know what’s going on it is so daunting to go searching for the answers yourself. While I’m not celebrating the Sjogren’s, nailing down a condition and a treatment is a triumph!

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  8. I’m so impressed with you! My strategy at this point is to learn to do the best I can, and maybe try again in another decade or so, to see if anyone has decided to develop a reasonable diagnostic process. Way to go!

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    1. Thank you! If I wasn’t so disabled, I think I would consider taking a similar approach. When I was healthier, I would try out new type of specialist every 6 months or so, have a bunch of (negative) tests, then I would go back to normal life and wait several more months before doing anything further. I’m grateful I’m still healthy enough to do research even if it is slow process compared to before.

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  9. What a coincidence! Did you happen to read my last post? The one thing the specialist in California wanted me to do is IVIG. I didn’t look into the price and, oh joy, I don’t have insurance. Well, I have insurance through the Affordable Care Act (thank god), but I’m sure there’s no point in trying to get IVIG covered if they won’t even cover my physical therapy.

    I never pursued Sjogren’s because my understanding is that there is no treatment except stuff for dry eye (I’m already on Restasis) and dry mouth, so what’s the point in the diagnosis. Maybe I’ll learn more about this from you.

    I think it’s so funny that we both did the steroids and now both need to find a way to do IVIG… I don’t think it’s too funny that we’re both exhausted and feeling sick all the time, though.

    Please share all you find out re IVIG success stories. You really are helping so many people! X

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    1. I did read it! Am I remembering wrong or did you say you tested as immunodeficient? If you are, immunodeficiency is one of the few on-label uses for IVIG. If you call your insurance, you can ask for a list of conditions approved for IVIG treatment to find out if you’d need to fight them or not. Does the Affordable Healthcare Act have enrollment periods which would mean you are stuck with the insurance you chose until the next enrollment period?

      Back in 2010, an immunologist (former owner of the clinic I am currently going to), “If we can get you approved for IVIG, I believe you will feel like yourself again.” However, because I wasn’t immunodeficient and we didn’t have any diagnosis whatsoever, we didn’t pursue it. I have read about it off and on as it pertains to Dysautonomia. Now that I have an autoimmune diagnosis, I plan to devote my life to stalking my insurance company and get them to pay for this.

      There is no cure for Sjogren’s, but there are things that can be done to lessen the severity of the disease and stop damage from continuing. The scarred tissue I will never get back from what I have read. Sjogren’s is very closely related to Lupus. I’m not sure if you know about Lupus treatment, but if you do, that may give you an idea of Sjogren’s treatments.

      It is a bit strange to think I need both immunosuppression (so my body stops attacking itself) and IVIG which is an immune booster, but I hope to explain why IVIG is thought to help in autoimmune diseases in a future post if I get energy. At the very least, I will post the research paper I write for my insurance company! I hope to have that done by the 29th.

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  10. I have a Facebook friend who has POTS and Sjogren’s and has been doing IVIG for a few months! It’s pretty early on in her treatment, so she hasn’t had much to report yet, but her doctor’s very hopeful.

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    1. Fingers crossed for your friend! I am not 100% convinced IVIG is the answer, but I think it makes a hell of a lot of sense to try and would like the chance to see if it will help me.

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  11. Yay! Finally some progress! I thought I had this, or diabetes insipidus, because I’m so dry all around. Even if you don’t get that big diagnosis right away maybe one doctor will take a look at this syndrome & fit the next piece to the puzzle. Baby steps are ok as long as you’re still moving! ๐Ÿ™‚

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    1. It had come to my attention several times throughout the past 2 years, especially thanks to a POTsie friend who has it, but I never thought it fit until recently when I realized it fits perfectly. Thankfully my friend was patient with me. ๐Ÿ™‚ Thank you for the reminder that even though I still have a mystery disease, at least I am closer to figuring it out how to restore my health than before!

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  12. I think I’ve talked to you about this before but my mom has systemic Sjogren’s Syndrome – seronegative (meaning she does not have the antibodies). She was officially diagnosed via lip biopsy but meets other criteria in other testing (like the eye tests). Plaquenil has helped her big time. It is believed that I have “early signs” of Sjogren’s (simply because I do not have enough of a lymphocyte focal score in lip biopsy). I took plaquenil for a short time just prior to my tilt table test confirming POTS. I had to stop the medication because I was taking several others at the same time and having poor reactions (one of them was Salagen which is another medication for Sjogren’s). I was at a breaking point at the time so I never tried to go back on anything individually.

    I’ve researched a A LOT about Sjogren’s for my mom and I was somewhat of a health advocate for her when she first got sick (they initially thought she had Rheumatoid Arthritis). My mom also has low immunoglobulins (specifically A & G). So if you ever want to talk about it and share what you’ve learned, I’d love to know! Even more so because of how borderline I am.

    I am so glad for you that you found another answer but I am also sorry for the obvious reasons. Hope you are getting some adequate rest (and also you had a great time wedding dress shopping!).

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    1. Interesting. I’m Jackie’s Mom and have been diagnosed with seronegative RA and have very low IgA levels. Do Sjogrens and low IgA go together? When Jackie was little had her tested multiple times for IgA deficiencies because of freq. kidney infections. My brother has IgA nephrapathy. Was diagnosed with JHS/HEDS because of Jackie’s research.

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      1. Jackie you should be a doctor with how many people you have helped get diagnosed and to all those you are helping point in the right direction. Fate put me in the hospital elevator with you for a reason and I am thankful to have met you ๐Ÿ™‚

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        1. Aww, you are very sweet! I’m grateful to have the opportunity to help people. When I had to quit working, I worried helping others find the resources they need to regain hope was over for me, so comments like yours mean a lot. ๐Ÿ™‚

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      2. I have wondered the same thing! I haven’t found official answers in research yet.. Though I will admit I haven’t been able to focus entirely on this portion yet. It’s very overwhelming as I am sure you know. I will keep you guys posted on whatever I find, for sure.

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      3. I just downloaded an article about the topic of immunodeficiency and autoimmunity co-occuring since, intuitively, they seem opposite. It explains how it is possible. I didn’t get to reading it yet, however it did say 20% of people with immunodeficiency (the fact I read was for primary immunodeficiency, not a selective immunodeficiency) have an autoimmune disease eventually.

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    2. I plan to post what I learn as I can and I hope it helps you. If you ever have any specific questions of if I have come across something in my reading (cannot guarantee I remember everything from every article of course), I am happy to answer! I have such a backlog of information to post, but when I have energy, I tend to do other things first.

      Is your ANA elevated? I didn’t know this, but mine wasn’t back in 2010 and with my symptoms, it should have been re-checked occasionally.

      If you do a Google search for “neurological symptoms of Sjogren’s” you should get quite a bit of hits. I was surprised in how many ways it can effect the body. It is similar (but definitely not identical) to Lupus in that sense. It is a disease known in the literature for attacking the nervous system, but all most doctors know is it causes dry eye and dry mouth. Dry eyes and dry mouth are the least of my worries!

      Wedding dress shopping was quite a bit of fun! It was exhausting, but it was nice to be exhausted from something normal people do instead of a medical appointment.

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  13. Hi Jackie. I just found your blog today. I love the way you put things! Well, I have “probable” Sjogren’s, having received this non-diagnosis two years ago after getting sicker for several years. Now I am being evaluated for POTS, and I’ve had these symptoms since 2010. I am very impressed at your persistence at pursuing diagnoses and treatment. Thankfully I have a rheumy who is treating me (Plaquenil) despite not diagnosing me. However, I suspect that the the autoimmunity is causing the POTS in my case. So I’m probably going to need a bit more than Plaquenil.

    Have you looked into biologic medications? I have seen some indications that they might be useful for autonomic neuropathies associated with rheumatic diseases. I am looking into that as well as IVIG. I think I may be developing a trigeminal neuropathy as well. I’m trying to get prepared for my November appointment. However, I’m pretty sure I would have to convince her to diagnose me before she would prescribe any heavy hitters. So, best of luck on your quest! I would be very interested to hear how it works for you. So much depends on the doctor’s views and communication.

    Cheers,
    Nicole

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    1. I have looked at the research on some of the biologics for rheumatological illnesses. At the time, my research was broad since I hadn’t zeroed in on my illness — technically I guess I still haven’t since I have a “lupus-like” illness too. Do you have your eye on a particular biologic? Certain ones look promising and have helped people, but I am unsure what I think of focusing on single cytokines found in inflammatory diseases. I cannot remember which cytokine a particular biologic drug inhibited, but I did notice the cancer rate of patients who had taken it/were taking it was too high for my comfort. It may have been a TNF inhibitor. Ilaris inhibits IL-1. I found IL-1 pretty interesting to learn about and thought perhaps Ilaris would be helpful, but still I am really hoping for IVIG!

      Have you had a lip biopsy performed to look for Sjogren’s? If not, look into it! It is a way to establish a diagnosis when a patient is seronegative.

      Good luck with your appointment in November and thanks for your encouragement!

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      1. I am not sold on any particular biologic. However, since my blood work shows high risk for RA, I am open to that. The great thing about IVIG would be to (maybe) get sick less often, rather than more often with a biologic. I am already sick too often as it is! And I do not know which is considered more effective for peripheral neuropathies. But, yes, I think it’s about time I pushed for a diagnosis. I am not crazy about getting a lip biopsy, though. Maybe if I go to a neurologist and show PN then my rheumy will commit to Sjogren’s. ๐Ÿ˜› Anywho, it’s PN and not Sjogren’s that qualifies a body for IVIG.

        Good luck to you, too!

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        1. Personally, I like to have the testing done to confirm certain diagnoses (or not). That way, I can keep an eye on the research to know which treatments are likely to help me and also to know if I should be looking for other explanations for my symptoms. From what I’ve heard, the lip biopsy usually isn’t too bad, but I definitely understand wanting to avoid it if you can; after all, that is exactly what in doing since I’ve had the lacrimal gland biopsy!

          I’m hoping to have my neuropathy confirmed via a tiny skin biopsy (ENFD and SGNFD) to build my case for IVIG. From what I read, on label use of IVIG for neuropathy includes chronic inflammatory demyelinating polyneuropathy (CIDP) and multi-focal motor neuropathy (MMN), but it is easier to convince insurance companies to pay for off label IVIG for other neuropathies also caused by autoimmune diseases than off label for an autoimmune disease alone.

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          1. I agree with you about diagnosis. In my case, I’m pretty sure that it’s Sjogren’s. My rheumy just won’t commit. However, as she is treating me regardless I’ve been ok with that. Now that I suspect neuropathy I’m thinking along the same lines as you – get it diagnosed so it can treated! Plaqunil is not going to cut it, that much is clear. So, I don’t which will come first for me, the Sjs or the PN dx. I am guessing that if I am dx’ed with PN then my rheumy may be more willing to commit to a diagnosis. However, because my bloodwork says “RA” and my symptoms say “Sjs” – and both can cause PN… well… we shall see. I think that you are on the right track with the biopsies. That may be the route I take as well.

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  14. Ivig? Very long treatment time, expensive per treatment, and sometimes comes with not great side effects! This seems like an off label use I’m guessing? Have you seen a hematologist? I haven’t heard of a rheumatologist prescribing this! Have you been to one? Remicade is amazing and I believe would be an FDA approved drug for your diagnosis.

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    1. It’s off label but some people with my condition (POTS caused/exasperated by Sjogren’s) have gotten their life back after starting it and there is literature out there about its use in Sogren’s. I am hoping to get neuropathy confirmed since some types neuropathy caused by AI disease are on label uses. I have the symptoms and having a confirmation will hopefully make it easier to make a case!

      Thank you for the suggestion. Personally, I would prefer IVIG over a TNF inhibitor, but I know they are helpful for many. IVIG or not, I will be on Plaquenil which hopefully will help too.

      Rheumatologists will sometimes prescribe IVIG if nothing else works for someone in my situation. I am seeing the rheumatologist the 29th to see if he agrees with the immunologist’s support of trying to get me it. When you consider I am 25, spend 90% of my life in bed, and well over 20 doctors have not been able to help me much, it makes it easier to see why such an extreme measure is taken with patients like me.

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  15. I just started plaquenil 6 weeks ago for sjogrens (fyi it causes major weakness for me so I had to take it only at bedtime, that helped). Between my neurologist and rheumatologist, they are keeping me on the ivig (my rheumatologist says they want the ivig treatments closer together but the neurologist has to do it since their the ones who originally prescribed it)since I do get some neuropathy, OI and hyperpots relief. The rheumatologist wants to start me on rituximab infusion also to see if it helps with the rest of my muscle and joint pain. As well as hoping it slows the progression. My new primary has also added butrans patch for pain relief. Like you …. Sjogren’s is systemic and now their checking for mast cell activation disorder. Personally, I just need to stop going to the doctors so they can’t add any more autoimmune disease.
    Glad to hear your still kicking…. Love the new tub.

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