Sjogren's Syndrome- Dry eyes, mouth? get this one ruled out, or in!

[MariaMagdalena]

Hey everyone! I was going to post this to the forum reserved for alternate or overlapping diagnosis, but there was no heading there for this so I guess I'm putting it into the general circulation. If I missed a more appropriate placement, feel free to move it, mods.

I have recently been making the rounds to different specialists in order to rule out everything else that could be going on with me either with, or causing, or other than CFS/ME. This was never done for me before (though I've been diagnosed with CFS for 8 years). The doctor I went to just went down the diagnostic tree and saw I fit and that was that. I have CFS. As I've worsened dramatically in the last year and a half, I realized I really needed to investigate fully what is going on and not settle for CFS as my fate until everything else could be ruled out.

This week I went to a Rheumatologist. I was fortunate to get a Rheumatologist who is very old (over 80) and thinks like doctors used to think (plus has a ton of experience).

The first thing I said when I met him was that I was here to just check all the boxes of rule-outs, that I fully did not expect him to find anything. He asked me a bunch of questions and when he started asking about dryness, I was like, umm. yes, yes, yes.. but I thought I was just weird about certain things. I had no idea that:

the fact that my eyes feel like pins are being shoved through them, blurring so bad I can't read through my bifocals regularly, and they burn constantly

I can barely sweat

I've had repeated instances of corneal abrasion while sleeping through many years

Before I got a humidified CPAP machine I couldn't sleep because of plugged nose as soon as I laid down the need to wake up and drink water many times a night.

I haul a water bottle everywhere I go and can't get in a conversation for more than a couple of minutes without sipping water the whole time.

I can't eat a meal. Period. without something to drink.

I'm like a vampire when it comes to the sun.

My teeth always have cavities and I've lost 3 of them

These are just a few things that are connected to one probable diagnosis.

Sjogren's Syndrome

All of these things have just been minor annoyances that I was just used to as "me" and they pale so much in comparison to debilitating, never ending "flu", and brain fog to the point where I feel like I must be borderline dementia.

Also I discounted all of these dry symptoms because I've been on antidepressants since I was 30, continuously and I assumed this dryness was all from that. I've pared the AD's down to only 1 tricyclic at this point, and thought the dryness shouldn't be as bad as it is, but still...

I asked if I needed antibody tests to confirm that I have SS and I loved what he said. He first told me of a patient who came to him after the University refused to diagnose her because she tested negative for the 2 antibodies they test for. She came to this doctor and he diagnosed her. He said, "How arrogant are we? Yes, we know there is antibody A and B. But what if there is a C and D and E that we just haven't discovered yet? I prefer to diagnose my patients the way good old Sjogren did back in the day, but listening to them and asking questions."

So, I don't know if have SS AND CFS/ME or if the CFS dx is wrong?

He put me on a medication called hydroxychloroquine (Plaquenil) which is a milder immunosuppressive. He said I may not see any improvement for 6 months.

But something quite shocking has happened to me in the 3 days I've been on it.

First, Know that at this point, I am homebound and any trips outside the house will put me into PEM. Even being on the computer too long, or taking a shower puts me in PEM.

Yesterday I went over my limit to go to a family birthday gathering. While there I got in a nasty row with my unstable adult daughter and the rest of the family attacked me for being "unkind". The fact that my daughter is off the rails these days and somebody needs to confront her was brushed aside. My "excuse" of being sick was just that. If I am so sick that I have to be unkind, then maybe I should have stayed home. Forget the fact that this woman is unraveling and has an autistic child to raise, and I can't help her. Oh. That's an EXCUSE again. I was told by her that I have no right to care about my grandson or say anything at all about his wellbeing because I never help with him and am just selfish. Not sick.

Ok... gone off topic, but I say all that to say this:

Today I have not crashed yet. I keep looking at the clock and expecting to come but it hasn't yet and it is not midafternoon.

I spent the rest of yesterday after the argument crying and yelling on into the evening. What happened combined with the relentless fatigue/malaise/brain fog miasma 24/7 for months just caved me in entirely. I just couldn't take it anymore and I wanted to die.

Maybe it is too soon to write this. Maybe it will hit tonight or tomorrow, but I SHOULD by all rights be bedbound now. I have been a hair's breadth away from it for the last month.

Maybe I should be devastated to be diagnosed with an incurable disease, but if this is for real it is better than having ME because at least they know some about it and there are available meds that can help.

This diagnosis needs to be ruled out!

Know also that it isn't simple to diagnose. Many times tests are normal. My inflammation tests all came back normal.

 

[Rufous McKinney]

This diagnosis needs to be ruled out!

I experience considerable dry conditions...which can at times be acutely terrible. I don't take any pharmaceuticals that include that as a symptom so mine is not a pill side effect.

This is the main reason I take chinese herbs: they prevent that dryness from getting extremely severe. And thats called a severe Yin Deficiency, in chinese medicine. (yang, heat, is therefore not being balanced, hello inflamed brain).Heat rises, so everything up top is boiling over one might say.

Meanwhile: lets hold the energy for you to Feel Better despite having relatives!

 

[chronic-tea]

OH MY GOODNESS @MariaMagdalena (Please note: I like to use all caps to show enthusiasm or just lots of feeling, not anger, so I am not yelling at you! I'm... feelings feels because our circumstances are so similar, and also excited for you that you have answers.)

THANK YOU for this post, as well as for flagging it for me on my "introduction" thread. I am surprised/a little "annoyed" that there isn't an area for autoimmune diseases because, especially in the case of Sjogren's, there is so much overlap and it's so easy to miss.

It's so easy, I think, for Sjogren's to get a CFS diagnosis if a doctor isn't asking about dryness (although there may be some evidence that neurological symptoms can develop before dryness!), thinking critically/out of the box, or even simply isn't very educated about Sjogren's. This last part is sadly too common- it sounds like you got very lucky with your rheumy! Seriously, SO SO SO SO SO LUCKY! GIVE HIM A GOLD MEDAL!

I asked if I needed antibody tests to confirm that I have SS and I loved what he said. He first told me of a patient who came to him after the University refused to diagnose her because she tested negative for the 2 antibodies they test for. She came to this doctor and he diagnosed her. He said, "How arrogant are we? Yes, we know there is antibody A and B. But what if there is a C and D and E that we just haven't discovered yet? I prefer to diagnose my patients the way good old Sjogren did back in the day, but listening to them and asking questions.".

This is, imo, how they should be doing it! We do not currently have any tests that rule-out Sjogren's if they are negative. We only have tests that can support a diagnosis if they happen to come up positive. Estimates are currently that at least 30% of Sjogren's patients are antibody negative.

This is also exactly my problem right now. I'm negative on the antibodies. I had a lip biopsy that did not show Sjogren's- but also did not have enough material for a "full evaluation." Still, doctors are calling it negative. Only after I scheduled and got the lip biopsy did I find, from the internet, that

1. Lip biopsies are actually poorly standardized, i.e. the quality of the procedure you get varies more from doctor to doctor than is typical. One of the most common issues that produces a false negative is not getting enough tissue (as in my case). They're also poorly standardized in terms of how pathologists interpret them; different pathologists often come to different interpretations.
2. Lip biopsies might only show up as "positive" during a specific window of the disease- but we have no good information on this. The information we do have suggests one can have significant Sjogren's symptoms for years before a lip biopsy turns positive. If one has it for too long though, the salivary glands can deteriorate to the point that they will again be a technically negative biopsy, but because they are so significantly damaged rather than "not inflamed enough."

So, in my case, I've rescheduled another lip biopsy with an expert in the procedure who I am lucky enough to have near me. However, I have to wait over a month for that because they're an expert in the procedure. Even so, it might turn up negative! I still believe I have Sjogren's.

I might have guessed that age could have been a hindrance in your rheumy's case, because my understanding is that a lot of old school thinking on Sjogren's is it's "just" dryness and only about treating those symptoms- when it fact, as it sounds like you are aware, it is a progressive autoimmune disease that causes multisystemic problems as serious as all the others. My doctors, including rheum, all know I have dryness, and yet I'm the one championing a SS diagnosis for myself/persuading them to take it seriously, only after I accidentally did the right kind of online research.

I am jealous you got Plaquenil! But so so so happy for you! That's currently my goal.

As you noted in my intro thread, we have so much in common. I meant to reply to your question ("what makes you suspect Sjogren's?") on that thread with some of what I'm going to say here. I feel bad now for not replying sooner so we could compare notes/I could push you towards looking more at Sjogren's- but I'm so glad you got to this diagnosis quickly anyway!

1. I also have extreme mouth dryness that I thought was just me. My mom has a tendency towards mouth dryness, I've always had a tendency toward it even when I was a kid, and I know some of the meds I'm on exacerbate it, so I've never thought too much about it.

Rant about a bad doctor, feel free to skip
My mouth dryness actually really annoyed my former PCP, who was terrible in lots of ways and complained more about my mouth dryness than I did (at the time, I wasn't complaining about it at all, because of the above). She and I would literally get into arguments about whether or not I was drinking water because my mouth was so dry. She didn't believe me that I actually drank a lot of water because my mouth was so dry, and kept telling me to drink more. I eventually calculated that most days I was getting 70-100 oz of liquid, most of it in the form of water, and of course she didn't believe me. She told me that I should give up my decaf coffee, herbal tea, V8, and everything that isn't water "there is no reason to drink anything except pure water," ever.

I'm sure you can make an argument that these things can exacerbate dry mouth due acidity or tannins or whatever but that's not the argument it felt like she was making- just that I was not taking care of myself . It makes me sad/angry though, because clearly in the dry mouth she saw a serious problem that I was dismissing. I wasn't complaining, but what she saw when she examined me set off alarm bells- that she then blamed on me, like she did every issue I experienced. She really missed a great opportunity to help me get diagnosed. She had diagnosed me with dry eye in addition to dry mouth. And I eventually started complaining about fatigue that got worse with activity/was interfering with my ability to exercise, and that I had ruled out mental health causes for (bless my therapist and psychiatrist for helping me with this). But still, my PCP told me that she was 100% sure there was nothing wrong with me because all my blood work was normal (she didn't even do the rheumatology tests, just the basic CBC, metabolic panel, and ruling out diabetes!), and that I should work with a personal trainer and my psychiatrist. I am still scarred from visiting her.
End rant

Only recently have I realized how severe my dry mouth has gotten. I had to sit through my friend's PhD defense in an auditorium in which there was absolutely no drinking, not even of covered water. It was 60 minutes long, and I loaded up on water beforehand- and it was painful. I was so uncomfortable. At the same time, I've been having trouble sleeping due to dry mouth. I don't want to drink a lot before bed because getting up to go to the bathroom is seriously impacting my sleep, but even so, there's no amount I can seem to drink that makes my dry mouth better. These helped me realize the dry mouth really was getting significantly worse than it was, say, 3 years ago, and that, this is not normal.

An aside, my husband doesn't drink water with his meals, and it practically hurts me vicariously to watch, I'm like, HOW ARE YOU DOING THAT DOESN'T IT HURT?!?!

2. I have severe dry eye- more severe than I realized.

While my former PCP was always nagging me about how dry my eyes were, and I was having a lot of itchiness/eye symptoms, I thought it was my cat allergies! (She never told me to see an ophthalmologist or told me the risks of having dry eyes, although she did tell me to use a compress.)

When my neurological symptoms started to get bad and include blurry vision, I made my first appointment ever with an ophthalmologist. They were scheduling like 5 months out, and I thought I'd be seeing them to make sure that there were no eye issues that were contributing to my blurry vision. Well, it turns out that the appointment happened right as I was like "omg, this might actually be Sjogren's," and sure enough, my eyes are dry in exactly the way that Sjogren's patients are (based on results from tear test and stain).

I also haven't been able to cry in... a long time. Sometimes I feel like crying and kind of do a dry wailing instead. I thought maybe I was... deliberately, yet also without my knowledge, being histrionic? For, like, pity from my husband? It didn't occur to me that physical inability to produce tears was, you know, a symptom.

3. Sjogren's is a leading cause of dysautonomia and small-fiber neuropathy.

Only once I realized this did I take it seriously as a diagnosis. Before that, I was like "maybe, if I have it, it could be contributing to the fatigue a little bit, but it's not the cause"- because so much of what you read online is about the darned dryness! Once I started searching neurological symptoms and Sjogren's, I was like OMG. THIS. IS. IT.

Sjogren's frequently causes autonomic dysfunction, which can cause:
-Severe fatigue
-Exercise intolerance
-Brain fog, mental confusion, etc.
-POTS (postural orthostatic tachycardia syndrome- I'm waiting on my tilt table test, but when I do the standing tests at home or in clinic, I'm positive every time)
-Small-fiber neuropathy, which can cause things like paresthesias (the burning, itching, pricking, cold, numb, and tingling sensations I've been getting everywhere) and doesn't show up on normal nerve/neurological exams
-GI dysfunction, including constipation, difficulty with food moving through the gastrointestinal tract at a healthy speed, GERD, and pretty much every other GI issue under the sun
-Heat intolerance

The problem is most neurologists don't know much about / aren't trained in / aren't even interested in autonomic dysfunction, or seem to think it could really happen, especially in patients with vague symptoms like "fatigue" and "nausea."

I'm currently waiting to see if an autonomic disorders clinic near me will accept the referral from my current PCP. My neurologists think I'm a headcase, although they were willing to order a tilt table test for me (and spend a great deal of time preparing me for the possibility that it will be negative). They also put my possible Sjogren's diagnosis entirely on my rheumy ("you have a rheumatologist who is dealing with that") / clearly don't think it affects the neurological picture. I am looking for new ones, but it takes time / I'm waiting to find out about the autonomic clinic first.

I know exactly what you mean about this diagnosis being maybe a positive thing. I was so excited when I "realized" I had it, I shook my husband awake! It's good to have a diagnosis that fits, that is in the medical textbooks (although sadly, not always accurately), and that there are treatment options for. It sucks that it's incurable, and it sucks that it's not as well-known about/researched as other autoimmune diseases, but it's something. There are things to go on in terms of treatment.

Now my struggle is getting that treatment without good biomarkers.

Interestingly, while it's not a first-line treatment, Rituximab is frequently used with success in Sjogren's patients. (Which reminds me- did you know that swollen lymph nodes can also be a part of Sjogren's syndrome?) Given that some research indicates that the neurological symptoms / dysautonomias can occur before dryness, and that even then patients often do not report dryness because the other symptoms are so debilitating it doesn't occur to them, I have to think that there are a great many people out there with Sjogren's who have been diagnosed with CFS. It's probably even worse when you consider that Sjogren's is often "ruled out" because of biomarkers that aren't specific/reliable enough to actually rule-out the disease.

I personally think that Sjogren's probably explains everything well enough that a CFS label is diagnostically "unnecessary" for me, but you could probably make the argument that the PEM/worsening of symptoms with exercise means that I could keep the CFS label for now based on us not knowing enough about these diseases? Part of me doesn't want to "give it up" because I think the CFS community is... for lack of a better word, precious. No seems to one talk about PEM in autoimmune conditions / conditions that aren't CFS. The CFS community has a language, a skepticism, a reach, and a spirit of advocacy that is just unrivaled. I know you can't "have CFS" because you want to keep the community, but,

THANK YOU FOR SHARING YOUR EXPERIENCE! I hope that Plaquenil continues to go well. Please keep us updated, and hit me up anytime!!!!!!!!!!!!!!!!!!

 

[kangaSue]

I've seen it crop up a few times now that an original ME/CFS diagnosis turned out to be Sjogren's some years down the track, such is the nature of the typical antibody set that can take a decade or more to throw up a positive result in Sjogren's (and Lupus for that matter).

Quest Diagnostics have a different antibody panel said to be earlier markers for Sjogren's, their 'Early Sjogren's Syndrome Profile' test, when the standard ANA and ENA panels aren't detecting positive results.
https://www.questdiagnostics.com/testcenter/BUOrderInfo.action?tc=93748&labCode=AMD
Can't find the reference paper offhand but another Quest paper said of this early panel;
[The current Ro and La antibodies can delay the diagnosis by over 6 years. Recently novel antibodies identified to salivary gland protein 1 (SP-1), carbonic anhydrase 6 (CA6) and parotid secretory protein (PSP) using western blot methodology. Further studies have shown that the isotype differentiation of the markers adds to the sensitivity of diagnosis of SS. These autoantibodies occurred earlier in the course of the disease than antibodies to Ro or La. In addition antibodies to SP-1, CA-6 and PSP were found in patients meeting the criteria for SS who lacked antibodies to Ro or La. Furthermore, in patients with idiopathic xerostomia and xerophthalmia for less than 2 years, 76% had antibodies to SP-1 and/or CA6 while only 31% had antibodies to Ro or La.
Antibodies to different isotypes (IgG, IgM & IgA of SP-1, CA6 and PSP are useful markers for identifying patients with SS at early stages of the disease or those that lack antibodies to either Ro or La.]

There can be a couple of things to cause dryness symptoms though, either that or they can be concomitant conditions and autoimmune conditions have a bad habit of attracting company.

Autonomic Neuropathy for one, which can be idiopathic or autoimmune. Small Fiber Neuropathy when it also affects autonomic fibers too is a very similar thing to AN (or they may be one and the same thing as the terms AN and SFN are often used interchangably in some ofthe medical literature).

Autoimmune Autonomic Ganglionopathy is the other thing that can cause severe sweat abnormalities, but I've seen plenty of instances where AAG and Sjogren's are concomitant conditions.

It's even rarer still to be antibody tested for AAG, but that's probably more down to there being few centres in the world that have an assay for the main antibody found in AAG, alpha 3 nicotonic ganglionic acetylcholine receptor antibody. Mayo's GID2 or DYS2 panels covers it though.

Mestinon is another med that tends to be used in any of these conditions to promote greater sweating or salivary production.

 

[chronic-tea]

Dysautonomia International claims that Sjogren's syndrome is the 2nd most common cause of autonomic neuropathy.

My understanding is that the latest expert thinking is a lot of SFN, especially in non-seniors with multi-systemic difficult-to-diagnose illnesses, is likely autoimmune in nature. Sure, idiopathic autonomic problems are still diagnosed (although it's also worth noting that many doctors are behind on autonomic issues in general), but I personally feel that autoimmune causes should be considered very strongly in cases of SFN where the cause isn't immediately apparent. In my opinion, an SFN diagnosis may in fact support a larger diagnosis of Sjogren's or at least of an autoimmune basis to a combined sicca complex/neurological symptom presentation.

Unfortunately, while the early panel's advantage is that it shows up "earlier" (before the other antibodies/even when they are negative), it isn't sensitive enough to be a "rule-out" (you can have Sjogren's and not test positive for them; it is still the case that Sjogren's cannot be ruled out with blood tests).

It is true that AAG can often look a lot alike Sjogren's. However, in cases where they truly do co-occur, it is likely that Sjogren's is causing the AAG, not that they are spontaneously co-occurring (just as SS is considered to cause SFN and other dysautonomias).

The bottom line is, Sjogren's syndrome is an autoimmune condition that commonly causes neurological problems. In fact, neurological symptoms often occur before dryness symptoms do.

 

[MariaMagdalena]

Quest Diagnostics have a different antibody panel said to be earlier markers for Sjogren's, their 'Early Sjogren's Syndrome Profile' test, when the standard ANA and ENA panels aren't detecting positive results.

I've seen links to Quest labs before, but I don't understand how it works. What it looks like is that they do more specialized tests, but that one would have to pay out of pocket for them? If so that's not an option for me on Medicaid.

I don't know enough about Sjogren's yet to know whether this would catch the disease better than the standard tests. Theoretically, it seems it would but I don't know.

The doctor I saw said he wanted to see my response to Plaqunil in 10 weeks before he ordered tests. If those antibody tests are negative, I'll bring this more detailed test to his attention. Thanks for sending the link.

 

[MariaMagdalena]

@chronic-tea Thank you so much for all of your good information! I appreciate it very much since I've only begun research into this diagnosis and you've obviously been at it for quite a while.

it sounds like you got very lucky with your rheumy! Seriously, SO SO SO SO SO LUCKY! GIVE HIM A GOLD MEDAL!

Yes, I did get lucky with this Rheumy, HOWEVER, I realize I will need to push for objective biomarkers of the disease if I am going to get other medical providers to take it seriously, or if I choose to do another round of application for SS disability. After all this guy is over 80. When he dies or retires (doesn't seem soon. He is sharper than a tack and has more brain power than I did at my healthiest) I will be referred to another Rheumy who is likely to not hold his particular opinion.

Well, it turns out that the appointment happened right as I was like "omg, this might actually be Sjogren's," and sure enough, my eyes are dry in exactly the way that Sjogren's patients are (based on results from tear test and stain).

I was going to ask about ophthalmology. I actually have been thinking I need to go and see them for months because of my eye concerns. I want to ask you: Is one of your eyes more affected than the other? For me, my left eye is much more severely affected than the right one. This is what has led me to suspect that something other than antidepressant dryness was going on. It seems that with a medication SE there would be a more even impairment, and that is how it used to be 5 and more years ago.

Since you have had that abnormal tear test, can't they diagnose Sjogren's from that?

Wow, you can't even cry?! I am not that bad. I can still cry, proven to me just this last Saturday with a family quarrel. I cried a LOT that day, but the next day I surprisingly did not crash into PEM, but I had much more trouble with dryness, and had an attack of joint pain, flu-like symptoms in the evening.

About the question of do we have CFS or just Sjogren's. I think its important to keep in mind that CFS is not a disease, per se, but it is a collection of symptoms and when one has enough of that collection of symptoms, they qualify for the diagnosis. You and I, we do still qualify diagnostically for ME/CFS because we do experience PEM as a predominant feature of our illness.

I think what we might say instead is that what we might have found is the causative agent BEHIND our CFS. It is well known that CFS/ME is caused by different things. I guess you could say the myriad symptom clusters are the same regardless of the bodily insult.

This is why I feel that finding the Sjogren's is very positive news. It sure is better than progressively degenerating from some unknown cause.

About the water bottle incident, I completely relate. I've been known to take huge purses into spaces that don't allow water in order to conceal my water bottle, I need it so badly.

And about night time dryness: Have you had a sleep study done? I have obstructive sleep apnea and sleep with a CPAP machine. Before I had that, I was like you, having my nose swell closed when I laid down and having to wake up and drink water all night long. Now that has stopped because the CPAP provides a source of warm. humidified air to breathe, and pressure to keep my nasal passages from collapsing. I have to use a chin strap though to keep my mouth mostly closed. Funny though, I still bring my bedside glass of water with me to bed every night as is my habit even though I rarely need it anymore.

Oh and before the CPAP I had periodic incidences of corneal abrasion while sleeping.

I sure hope that someone diagnoses you soon and gets you started on some medication.

Lets stay in touch.

 

[chronic-tea]

@chronic-tea HOWEVER, I realize I will need to push for objective biomarkers of the disease if I am going to get other medical providers to take it seriously, or if I choose to do another round of application for SS disability.

Ugh, yes. That's the boat I'm in too- even if I find a rheumy who will treat, it would be infinitely better if I can get a biomarker so that future medical providers will treat (or even current other specialties accept that Sjogren's might be an underlying cause of my other issues) as well as for disability eligibility.

The scary thing is, any given biomarker could come up negative, which could then also be used "against" us in those cases where we are claiming (to providers or to disability) that we have Sjogren's. For this reason, I am personally unsure about pursuing the early Sjogren's test. Part of me really wants to because, if it's positive, that's so helpful! If it's negative, though, how am I ever going to get anyone to believe I have it?

This is also why it's essential, if one pursues a lip biopsy, to get someone who is an expert in performing the procedure and to make sure the pathologist they send it to is also an expert in reading the biopsy. That was part of my problem- I went to any old ENT. The recovery was painful and my lip still doesn't feel 100% normal. In the end, the biopsy showed some inflammation but without a high enough focal score to be positive. The fact that the pathologist said it wasn't enough material for a full evaluation didn't stop the doctors from calling the biopsy negative.

@chronic-teaI was going to ask about ophthalmology. I actually have been thinking I need to go and see them for months because of my eye concerns. I want to ask you: Is one of your eyes more affected than the other? For me, my left eye is much more severely affected than the right one. This is what has led me to suspect that something other than antidepressant dryness was going on. It seems that with a medication SE there would be a more even impairment, and that is how it used to be 5 and more years ago.

Since you have had that abnormal tear test, can't they diagnose Sjogren's from that?

One of my eyes is more severely affected the the other, based on the results. (Again, I'm just a really poor reporter of my own eye symptoms!) My results were:
-Schirmer’s test: <5mm / 7mm in 5 mins
-Dye disappearance test <3 sec/normal
-Punctate epithelial erosion noted in both eyes (supposedly these are "evidence of ocular surface dryness")
-Meibomian gland dysfunction both eyes (these glands are not secreting enough oil, which means the tears I do produce evaporate too quickly).

I've been told that these differences between eyes are common in Sjogren's, and that these tests support a Sjogren's diagnosis.

I would say, if you are currently being treated for Sjogren's, RUN, do not walk, to an ophthalmologist. If you have trouble getting an appointment, tell them you've been diagnosed with Sjogren's and have had symptoms with no ophthalmological treatment for years, and that your doctor is worried about your cornea health (even if your doctor hasn't said so ). If they still can't get you an appointment in a reasonable time frame, ask if the doctor will tell you what to do in the meantime to prevent eye complications from Sjogren's.

I've read that all Sjogren's patients should have a rheumatologist, a dentist (preferably familiar with Sjogrens), an ophthalmologist, and a gynecologist if applicable. At Johns Hopkins' Sjogren's Treatment Center (which I don't live near but love reading about lol), they also have neurologists for those with neurological symptoms (common) in addition to their gynecologists on staff.

It turns out we can get pretty serious complications from the dry eye, including cornea damage. I was told to use preservative-free artificial tears at least 4x a day, a nighttime ointment that helps with oil gland dysfunction, and a hot compress on my eyes for 10 minutes daily. It kind of freaked me out that they felt the dryness needed so much intervention and could be so risky. I hadn't been taking it seriously.

Sadly, the eye tests support a Sjogren's diagnosis but are not sufficient to diagnose Sjogren's, since there are other conditions that can cause dryness. *scream into the void * Granted, I don't seem to have any of those conditions, but . So even with eye exam, providers have to utilize the full clinical picture to reach a Sjogren's diagnosis, which means they need skill, Sjogren's-specific education, and good clinical judgment.

My current favorite Sjogren's resource is the blog sjogrensadvocate.com. It's run by Dr. Sarah Schafer, a Sjogren's patient who has an MD and who had a lengthy career in public health prior to becoming ill. Her personal experience as well as her familiarity with the Sjogren's research and the current challenges of getting adequate treatment for Sjogren's is incredibly enlightening and helpful.

She has an entire section on Diagnosis. This includes information about the 2016 Classification Criteria for Sjogrens, as well as their limitation as a diagnostic tool in the clinical setting rather than research purposes. I will not meet the classification criteria until/unless I get a positive lip biopsy or my SSA/Ro shows up positive. Dr Schafer also did not meet classification criteria of 7 years of her illness, and gives some details of her diagnostic journey on the Classification Criteria page.

I've found her section on Lip Biopsy particularly helpful, and highly recommend that anyone considering or scheduling a lip biopsy read it. Dr. Schafer had 3 lip biopsies: the 1st showed inflammation without a high enough focus score (needs to be 1 or higher) to meet Sjogren's criteria (negative result), the 2nd (7 years later) was positive with a focus score of 1.5, and the 3rd (2 years after that) showed no focus score because the salivary glands were too atrophied (advanced salivary gland damage caused by Sjogren's) which is still technically a negative result. Scary for those of us who need "proof" of Sjogren's!

The blog also has good information on Dysautonomia and Small-Fiber Neuropathy in Sjogren's.

Another good resource is https://www.sjogrens.org by The Sjogren's Syndrome Foundation. It is an amazing organization that has done essential advocacy work on a shoestring budget and is contributing to real change. However, I've found their website to be more focused on dryness and less focused on the other complications than I would like (although they are emphatic in their acknowledgement that the other issues exist!). The results of their Living with Sjogren's Harris Poll are incredibly enlightening in terms of what this disease actually "looks like" for those living with it. They also have useful brochures and resource sheets for coping with the various issues.

Smartpatients.com has a section for Sjogren's conversation also. Dr. Schafer is an active user there.

Dysautonomia International is focused entirely on dysautonomia, but I frequently find resources there specific to dysautonomia caused by Sjogren's. The president of that organization, Lauren Stiles, has autonomic neuropathy, including POTS, caused by Sjogren's syndrome.

Re: sleep, I had an in-home sleep study done. My understanding is those do best for moderate/severe sleep apnea, and they didn't find anything. I did get a trial CPAP, but it didn't seem to be measuring apnea episodes at a rate that is clinically significant, so I returned it. At the time, I was also deteriorating majorly health-wise, so it was clearly not the "cause" of my fatigue. I sometimes wonder if I did the right thing though.

@chronic-tea T
About the question of do we have CFS or just Sjogren's. I think its important to keep in mind that CFS is not a disease, per se, but it is a collection of symptoms and when one has enough of that collection of symptoms, they qualify for the diagnosis. You and I, we do still qualify diagnostically for ME/CFS because we do experience PEM as a predominant feature of our illness.

I think what we might say instead is that what we might have found is the causative agent BEHIND our CFS. It is well known that CFS/ME is caused by different things. I guess you could say the myriad symptom clusters are the same regardless of the bodily insult.

This is why I feel that finding the Sjogren's is very positive news. It sure is better than progressively degenerating from some unknown cause.

This is so well-said. It is exactly how I feel. Thank you.

Absolutely, let's stay in touch!

 

[GlassHouse]

I test negative for Sjogrens antibodies and even did a lip biopsy which was negative, but I have severe progressive dry mouth and eyes. My eyes are degenerating at a fast rate (the ophthalmologist said I’m producing abnormal proteins in my tears and none of the right proteins. My tears are like jello, or at its worst like hard rocks or sand).

Oh and dry vagina that’s fun.

I have tested low positive a few times on the early Sjogrens antibody panel. It’s hard to say if it’s playing a part or if the dry everything is due to all the viral and bacterial infections I have.

It’s definitely worth ruling out or in, which is why I pushed for the lip biopsy. But I guess it’s quite complicated to rule out since I was told that even a negative lip biopsy doesn’t necessarily mean you don’t have it.

I can’t be on anything immunosuppressive because I have an active brain infection but I just started low dose subcutaneous immunoglobulin G (insurance won’t cover the high dose for autoimmune conditions but I managed to get into one of those programs that will let you try the low dose for one month).

I don’t feel any different so far but I’ll update again at the end of the month.

 

[wigglethemouse]

Quest Diagnostics have a different antibody panel said to be earlier markers for Sjogren's, their 'Early Sjogren's Syndrome Profile' test, when the standard ANA and ENA panels aren't detecting positive results.
https://www.questdiagnostics.com/testcenter/BUOrderInfo.action?tc=93748&labCode=AMD
Can't find the reference paper offhand but another Quest paper said of this early panel;

I don't know enough about Sjogren's yet to know whether this would catch the disease better than the standard tests. Theoretically, it seems it would but I don't know.

I was negative for SSA and SSB but positive to 3 of the antibody tests in the Quest Early Sjogrens panel. That led to a skin biopsy that showed highly postive small fiber neuropathy which also occurs in Sjogrens. Quest should be able to give you a quote if you call them with the test number.

This Dysautonomia International article states that SSA and SSB can appear 10 years after the early markers appear.
https://www.dysautonomiainternation...sjogrens-antibodies-in-dysautonomia-patients/

 

[MariaMagdalena]

@chronic-tea Thank you for your post with so much good info and links! How are you doing now?

I've been on the Plaquenil for over a week and I am noticing a difference. I have been able to come out of the seemingly permanent crash state (the forever flu) I've been in for months. Of course, the first day I felt almost normal (the CFS good day normal, not the "back to work" normal) I went to church, sang in the choir, and played an intense video game for 4 hours. I guess I was hopeful that this med had cured my PEM and wanted to test it. I did crash 24 hours later, in the evening, but next day was not as bad as I would have expected and today, so far, I feel back to normal, so yes, there is significant improvement in this short of time.

What I wonder is if that is diagnostic in itself? I kind of wonder if the doctor prescribed it for just such a reason. Would someone who doesn't have Sjogren's respond to Plaquenil? Maybe that is a thing to ask for from a Rheumy, just a trial of plaquenil instead of constant fighting for "proof"?

A couple of things I've been wondering about lately also are, 1. I seem to have developed swollen salivary ducts inside my mouth. They're not painful. I'm not sure if I've had them before. There is swelling under my tongue and on both inside cheeks in the same place, too high for me to have bitten it. I've also got this weird pain in my neck, next to my trachea that comes and goes that I wonder now is a salivary gland pain? Has anyone had this?

2. Dysautonomia and SNF This is something I've been thinking a lot about recently. I even had a dream where I saw a doctor who did tests and diagnosed me with a version of this. I've been wearing a heart monitor for awhile now and I really don't think I have full on POTS as my HR doesn't jump way up just from standing up, but if I am heading into a crash it will go up quite a bit with just walking around the house.

I am wondering if it is the addition of dysautonomia that causes PEM? I have read other Sjogren's people talking about how good exercise is for them, but I can't get to where I can tolerate it without crashing.

With regard to SNF, I have another problem that concerns me. It has to do with gut action. Beware TMI grossness follows................

I have terrible problems with constipation that I have to manage quite aggressively. There are times when stool consistency is soft, but my bowels just are not responding. There is no reflexive urge response and I have to do all the work to have a BM and sometimes digitally remove it. This kind of scares me and I don't know what doctor to ask to see about it. I am wondering if it is part of SNF?

I do have pelvic floor issues that led to surgery last year, but this seems more to do with the smooth muscle of the colon/rectum, not the surrounding pelvic floor muscles.

Oh, and about that. This illness has accelerated SO much since that surgery where I had a hysterectomy and and Oopherectomy (they took my uterus and ovaries). Estrogen replacement therapy failed because it triggered migraines. I thought I read somewhere that lack of estrogen can trigger, or worsen sjogren's? Has anyone come across this info or know more?

I can’t be on anything immunosuppressive because I have an active brain infection

@GlassHouse Ooo that sounds serious! How were you able to determine this? What are/your brain symptoms? I hope it resolves soon.

I was negative for SSA and SSB but positive to 3 of the antibody tests in the Quest Early Sjogrens panel. That led to a skin biopsy that showed highly postive small fiber neuropathy which also occurs in Sjogrens.

Did you have to pay for the quest test out of pocket? Did a doctor order it? I am on Medicaid so probably not an option for me.

 

[wigglethemouse]

Did you have to pay for the quest test out of pocket? Did a doctor order it? I am on Medicaid so probably not an option for me.

At the time in 2018 I called my insurance and they told me their negotiated price was $143.10, and I would pay my copay % of that amount. When I got to Quest and they itemized the bill that item was the only one they couldn't confirm the price for. The doctor ordered the test.
https://testdirectory.questdiagnostics.com/test/test-detail/93748/?cc=MASTER

I very often find the insurance negotiated price is 5-20% of the list price, so if you definitely want to check. Don't want to pay 20x.

 

[Wishful]

Would someone who doesn't have Sjogren's respond to Plaquenil?

There's so much we don't know about how the body works, so yes, any drug could have a benefit for someone without actually having the disease the drug is meant to target. So no, it's not proof of having a particular disease. If other treatments meant for that disease also work, then you can be more confident about that diagnosis.

 

[MariaMagdalena]

I watched an amazing lecture yesterday on the topic of Small Fiber Neuropathy:

Anne Louise Oaklander | Small Fibers, Big Pain || Radcliffe Institute

In it she talked about how people with this disease that they are only beginning to understand could be misdiagnosed with Fibromyalgia. Almost every screwed up thing I experience in my body could be explained by this.

It is encouraging that this is new and being researched and treatments are tried with success, but discouraging that I will probably never have access to any of it. That being said, I really liked the slide that talked about what patients can do for themselves to improve the dysautonomia symptoms:

• Stand up slowly, esp. after eating or using the bathroom
• Lots of fluids and salt to raise blood pressure
• Regular exercise to strengthen heart and other muscle, to build more capillaries and mitochondria (yeah riiight)
• Elevate head of bed with bricks
• Compression stockings, abdominal binders
• Improve tissue oxygenation (no smoking, treat artherosclerosis)
• Avoid low-oxygen environments (flying, high altitude)
• Medication options include Midodrine, Fludrocortisone
• Rare patients need continuous IV hydration with saline

If you live on the East Coast USA you have a lot more resources. Can't find any in Seattle. Closest to me is Portland and since I have state Medicaid that's off the table.

 

[ElmAve]

Sorry I can't read all above right now but want to pass on important info re: plaquenil. I have SS (diagnosed via ~50 blood tests to rule out other possibilities) and have been helped by plaquenil for the past five years as my fatigue worsened - it is generally ineffective in regard to fatigue but helps with fevers, joint pain, etc. and may slow SS progression. BUT IT IS A HIGH RISK MED. Do not ever overdose as toxicity causes bad effects. Dose should be correlated to weight and is very rarely more than two tablets per day. I take only 1.5 as I am 125 lbs. You MUST see an eye doctor for an extensive "plaquenil" exam at least annually as plaquenil can permanently damage eyes - an eye doctor familiar with SS will know about this. Many people can't tolerate plaquenil, but it helps many others, and is used to treat other autoimmune diseases such as lupus.

I think in my case SS triggered ME/CFS. I am disabled by fatigue and orthostatic intolerance, but SS is for now pretty stable. Hope things are going well for you!

 

[kangaSue]

In it she talked about how people with this disease that they are only beginning to understand could be misdiagnosed with Fibromyalgia.

There are a number of articles and studies in the medical literature (including by Anne Oaklander) which conclude that Fibromyalgia as a general diagnosis can be wrong in at least 50% of cases and it's actually Small Fiber Neuropathy causing the symptoms

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3845002/
https://www.openaccessjournals.com/...t-of-fibromyalgia-or-a-separate-diagnosis.pdf
https://www.ncbi.nlm.nih.gov/pubmed/30238382
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5845259/
https://www.healthrising.org/blog/2017/12/24/fibromyalgia-small-fiber-neuropathy-symptom-study/

 

[chronic-tea]

@chronic-tea Thank you for your post with so much good info and links! How are you doing now?

Sorry I've been quiet for a bit, I was out of town! Now I'm preparing to move (Monday) and for my tilt table test (Tuesday). So. Much. Stress!!!

While I was away, I realized that if I drink 90-100 oz of fluid per day, and quickly drink 8-16 oz when I'm symptomatic, my POTS is much more manageable. So that strategy, combined with continuing the heart rate pacing, has allowed me to increase my physical activity without increasing significant PEM. I still had a couple of days where I had to rest for 1-2 days after exertion, but I was exerting myself a lot (for me, not for a "normal" person!) and it was exciting to be so "functional." I can now cook mostly without my office rolling chair, and I am now able to go on short walks again as long as I go slow. So I feel what you're saying about your new-found normal.

The bad news? I've been doing this so many days now, and combining it with consuming 3-5g of sodium per day, and it's helping so much... that after 3+ months of being POTS-y as all heck, I don't always reliably demonstrate a POTS response! And when I do, it's often very "mild", going from 65 to 98 bpm compared to the than 67 to 125+ bpm I've been dealing with that has probably contributed to my significant increase in disability (that none of my doctors believe ).

In fact, the past 2 days I've been frantically cutting back on liquids (to like 68 oz per day lol) and sodium, and only after 58 hours of this am I regaining my POTS-iness. I also turned the heat in my apartment up because I think the cool 65-68 degrees was contributing to my better symptoms. I think I'm going to "keep myself as symptomatic as possible" until the test on Tuesday, because I cannot afford to test negative.

Pictured Above: My actual neurologists discussing how my tilt table test is going to be negative and therefore support their current diagnosis of conversion disorder.

In addition to "needing" my POTS to be recognized and to work with a medical provider on treatment (even if lifestyle treatments end up being the most important), and to turn up a measurable testing irregularity to counter the "conversion disorder" diagnosis, coming up positive for POTS will allow me to be seen by an autonomic dysfunction clinic. Which will maybe be more open to evaluating me for small-fiber neuropathy. (My current neurologists- again, pictured above - don't want to test me for it because "over-testing carries its own risks," like reinforcing the idea that I'm sick, and "small-fiber neuropathy wouldn't explain the fatigue or the brain fog" ).

Needing to stay super symptomatic thoroughly sucks right now with my upcoming move. It's going to force my husband to do a lot more of the packing, etc. than I would like (which was always going to be the case, but I thought I'd be able to do more with my new-found water-drinking super power!). But even he agrees that being symptomatic for this test is like, the most important thing right now.

I feel you when you say:

I've been on the Plaquenil for over a week and I am noticing a difference. I have been able to come out of the seemingly permanent crash state (the forever flu) I've been in for months. Of course, the first day I felt almost normal (the CFS good day normal, not the "back to work" normal) I went to church, sang in the choir, and played an intense video game for 4 hours. I guess I was hopeful that this med had cured my PEM and wanted to test it. I did crash 24 hours later, in the evening, but next day was not as bad as I would have expected and today, so far, I feel back to normal, so yes, there is significant improvement in this short of time.

It's also so weird isn't it- how we always have to clarify, including for ourselves, "wait, this 'good day'/'normal' still isn't fully NORMAL normal." (At least, I always have to say this and remind myself of this, because I'm like "wait, why am I not working? I can now walk for 5 minutes at a time! Doesn't that mean I'm healed?")

I do personally think it's diagnostic. If you didn't respond, especially in the long-term, that would probably be diagnostic also. The fact that you are responding, to me, at minimum, suggests an underlying auto-immune cause.

My understanding is that, among smart rheumatologists, this isn't even that unusual: "You match clinical presentation even without the blood work, so let's see if you respond to treatment and if so, that's indicative of the diagnosis." The bloodwork/biomarker tools for diagnosing autoimmune conditions are so poor (leaving 10s of percents of patients out), I think this makes sense! I recently learned that this was how my father was diagnosed with rheumatoid arthritis 30+ years ago- and he ended up responding to treatment and also definitely having that condition (it's now visible on x-rays, etc).

Of course, as others have pointed out, Plaquenil and rheumatological drugs are not things to be taken lightly or on a whim. They do carry risk, and they require appropriate medical monitoring. But, for me, when we are already so disabled, it's worth trying the drug to see if it helps when the clinical presentation is indicative and so significantly interfering with life.

One could potentially make the counterargument to all I've just said that, because Plaquenil is originally an anti-malarial, it's secretly fighting some latent unknown infection and/or parasite, but I just personally believe the autoimmune theory is more likely/compelling/powerful.

A couple of things I've been wondering about lately also are, 1. I seem to have developed swollen salivary ducts inside my mouth. They're not painful. I'm not sure if I've had them before. There is swelling under my tongue and on both inside cheeks in the same place, too high for me to have bitten it. I've also got this weird pain in my neck, next to my trachea that comes and goes that I wonder now is a salivary gland pain? Has anyone had this?

I haven't, but I feel like I've heard of Sjogren's patients having it. It sounds Sjogrens-y to me.

2. Dysautonomia and SNF This is something I've been thinking a lot about recently. I even had a dream where I saw a doctor who did tests and diagnosed me with a version of this. I've been wearing a heart monitor for awhile now and I really don't think I have full on POTS as my HR doesn't jump way up just from standing up, but if I am heading into a crash it will go up quite a bit with just walking around the house.

Have you done a "poor man's" tilt table test? (Lay down for 5 minutes, stand perfectly still for 3-10 minutes and see what the heart rate does?) Sometimes it takes a few minutes for a full POTS response to flare.

There are so many forms of dysautonomia that it's perfectly likely and possible that you don't have POTS but do have another form(s) of dysautonomia, I'm just curious.

I am wondering if it is the addition of dysautonomia that causes PEM? I have read other Sjogren's people talking about how good exercise is for them, but I can't get to where I can tolerate it without crashing.

This is my personal belief. It seems the limited knowledge we have of dysautonomia indicates that it takes a major toll on energy, especially on exercise tolerance, cognition, etc., and I agree that while Sjogren's is fatiguing, it seems like without this piece, exercise helps the fatigue rather than results in the kind of crashes that those of us with PEM experience.

That being said, many of these patients are also probably on appropriate Sjogren's drugs which help (but which also maybe stave off or delay neurological complications), so that may be a factor too. (Plaquenil takes up to a year to have a full effect, I've heard.)

I have terrible problems with constipation that I have to manage quite aggressively. There are times when stool consistency is soft, but my bowels just are not responding. There is no reflexive urge response and I have to do all the work to have a BM and sometimes digitally remove it. This kind of scares me and I don't know what doctor to ask to see about it. I am wondering if it is part of SNF?

This could definitely be a pelvic floor issue. (My GI doctor told me that they treat constipation that doesn't seem rooted in stool-consistency at a pelvic floor clinic.) However, it could (in my view) just as easily/also be a part of SFN. Small-fiber nerves control the GI tract, and GI issues are major among people with autonomic and small-fiber issues. I think exploring an autonomic cause for this is very much worthwhile. (Also, are we sure that pelvic floor issues aren't sometimes caused by SFN? I truly don't know either way.)

I struggle with constipation too. I have to supplement with magnesium for it and watch my diet and stress, and, like you, it's completely unrelated to stool consistency. It's not quite as severe as you're describing, but I can definitely understand what you're talking about. And a year ago, I did have a very severe episode that I had to resolve with a bottle of magnesium citrate because no amount of fiber or miralax would help- I ate nothing but apples and beans for a week to no avail! (This also involved some digital removal, you're not alone!)

My GI doctor hasn't been super helpful in general because she doesn't seem very interested in the "whole picture" (i.e. that this isn't a "GI" issue in terms of "treat with fiber"- which I get plenty of- but is likely a neurological issue).

Oh, and about that. This illness has accelerated SO much since that surgery where I had a hysterectomy and and Oopherectomy (they took my uterus and ovaries). Estrogen replacement therapy failed because it triggered migraines. I thought I read somewhere that lack of estrogen can trigger, or worsen sjogren's? Has anyone come across this info or know more?

I don't know a darned thing about this, but I had my left ovary removed due to torsion and surgery on my right ovary to remove a cyst. My Sjogren's got much worse starting after the second surgery (even though it didn't involve removal of the second ovary).

 

[chronic-tea]

@MariaMagdalena

Good news for me! My rheumatologist has agreed to put me on Plaquenil regardless of the outcome of my second biopsy.

Also tested positive for POTS!

 

[kangaSue]

I watched an amazing lecture yesterday on the topic of Small Fiber Neuropathy

Come across this very informative paper about SFN just the other day
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5912271/

 

[MariaMagdalena]

@chronic-tea That's great! How are you responding to it? I've been on it 6 weeks and it has improved my fatigue so much and has kept me from crashing so much. I've been able to return to exercise, cautiously. However, it has caused these horrible itch attacks. It feels like pin pricks or insect bites but over and over. It does respond to benadryl cream though, so I can probably endure it since it helps the fatigue so much.