I read two articles recently that both said pretty much the same thing and I'm curious to know if anyone has ever heard of this before or if they have any other information. These articles talked about The 3 Stages of Sjogren's. I expected this to be similar to Stages of Grief or some such, instead what I found was an article that screamed: this is real, this is how it works. Next I thought- does my doctor know about this, and if so... why haven't I been told?
Here's a link to the article I thought was more professional:
It appears that it was perhaps originally written in Japanese and was published in 1995
NCBI just has an abstract from the full article, I'd like to read the whole thing myself. Basically it says that:
Stage I is glandular
Stage II is extraglandular (skin and such affected)
Stage III would involve extraglandular and lymphoid malignancy
These stages can last 10-20 each years (the article suggested this as an approximate, not an absolute in my opinion- and since there are individuals that have lived with SS for 30 or more years and don't seem to have ever gone past "stage I" I personally don't accept that certain year ranges can even be applied. The other article suggested that the stages don't necessarily present in order.)
This makes a lot of sense to me and I believe helps to classify some of the various effects it seems to be having on each of us. I'm thinking also that if this Stage system is utilized (if it isn't already) by doctors that maybe it will help with everyone getting past the idea that we just have dry eyes and that we're a little tired.
Hoping to hear your opinions and if any of you have heard more about this please let me know- I've hit a wall in trying to research it myself. Thanks everyone.
I read this when you posted it. It’s an interesting idea.
You questioned whether doctors are using this staging system. What I’m wondering is how would it impact treatment, especially since the length of the stages is so variable.
And then I was thinking. . . . For those people who have Sjogren's as a secondary disease, and not a primary, does this apply? For example, my primary diagnosis is psoriatic arthritis. I already had extraglandular involvement when I was diagnosed with Sjogren's. Since then I've developed small fiber neuropathy. Related to the Sjogren's? Related to the PsA? Who knows?
I doubt it would impact treatment as much as it might impact awareness. My doctor in particular during our first meetings told me many of the things we read here, such as: you have nothing to worry about as SS doesn't progress to be anything worse it's just dry eyes etc... When I pointed out that it was systemic and could affect my organs, she attempted to assure me that SS doesn't do that.
As for the length being variable, that's true with a lot of illnesses, I just felt that the way the article described SS in stages would provide a better understanding of it as a whole.
Interesting. I personally believe I should be diagnosed with Stage II myself and that my grandmother should have been listed as Stage III... instead my grandmother, my mother, my aunt and myself are all listed as having Primary Sjogren's even though we all have other illnesses as well (arthritis, raynaud's etc...).If you were indeed diagnosed with Stage II and not Secondary (which according to my research is a term which should not be used and yet is found all over medical texts) I'd love to know your Dr.'s name and location- could you PM it to me?
The concept of Sjogren's being a Secondary disease is sort of mislabeled. If your Sjogren's was dx's first would your PsA then be Secondary? That's one of the problems with the terminology- it comes down to which one was first to many doctors, others seem to believe that they have to show up in a certain order or they would both be listed as Primary or both as Secondary since there are overlaps as well as the simple fact that if you have a second illness then both are secondary to one another. I read an article some time ago that actually stated that Sjogrens got the designations of Primary and Secondary to assist with clarifying research and that it was not intended for use as a diagnostic listing and the Primary vs Secondary even caused some confusion in the research due to overlapping and almost simultaneous double dx's.... during your same office visit you could have been told that you have PsA as well as SS- in which case how would/should you be labeled. Regrettably the terminology has remained. My rheumy is absolutely convinced that I have Primary due to her reasoning that for me to have secondary I would have had to have at least 1 of three other illnesses at the time I was dx'd or that I'd been dx'd previously with one of them. I knew exactly which textbook she'd gotten her listing from and told my husband later that my doc needed to read the whole paragraph... the illnesses listed were examples. I then pointed out to my husband another medical text listing which showed 2 of my illnesses which weren't in my docs listed examples, and this text stated that if I had 1 of them that the Sjogrens should be recorded as Secondary. In your case since you already had extraglandular activity you would have started your SS journey with Stage II- doesn't change your treatment, and you could remain at that level, but you would lose the confusion of - is it Primary or not.
Secondary could probably be used to show that you have more than 1 illness, but this makes it sound as if it's in second place and that the other is more important. With the Stages concept it doesn't list: Primary Sjogren's Stage 1 (it could if SS is all you've got, otherwise enter the confusion again)... instead it lists: Sjogren's Stage 1. In other words: patient has Sjogrens and it is in its first stage. I think it would be great if all illnesses were more accurately described.
Has your doctor classified your Neuropathy as Secondary? Primary? Probably not.... so I ask you- why is SS listed that way?
(Sorry if this is too lengthy or scattered, and hopefully it doesn't sound mean at all... there's all kinds of thunder and lightning at the moment and my attention is a bit divided- but I wanted to respond to your posting tonight).
Not too lengthy or scattered at all. This is a really interesting discussion. By this logic, which does make sense, I would be classified as Sjögren’s stage 2, due to the neuropathy primarily. The neurologist said it’s classic for Sjögren’s.
Well, it's official, and apparently has been for a long time- I have Stage II Sjogren's (my rheumy and I still disagree as to whether it's Primary or Secondary). My rheumy seemed a bit confused by the term: Stage (at first), but agreed with the progression and informed me that in their write ups of disease classification she just uses: Primary Sjogren's with Extraglandular activity when submitting my paperwork. I told her about the articles (I've found more- if anyone is interested the best way to 'google' it is: Stage Three Sjogren's, this seems to zero in the search parameters and I ran into a whole lot of articles) and she said that she hadn't heard it listed quite that way before but it sounded right and that she would look it up as well.
So- I say let's get the word out there. Stages to me just makes more sense, and maybe it will be helpful in educating some of the new people that end up taking this journey.
The "stage system" has never been adopted as it indicates that the syndrome follows a particular of progression which it does not. Keep in mind the Sjogrens is NOT a disease but rather a Syndrome. There is a difference despite the fact we ofthen use the more common "diseases" More common would be what we are familiar with being SICCA, Glandular, Extraglandular etc.
Along with that the Terms Primary or Secondary have less to do with the syndrome than they do with with defining patient cohorts for research. Primary meaning the patient has SS and little else and Secondary meaning SS coexists with other diseases. It has nothing to do with severity of the disease as one would be constantly redefining whether ones Diseases were secondary or primary. I think any of us who has other diseases can identify with that.
As research in to SS has moved more into the hands of the immunologists, the distinction has become less important. Most patient advocacy groups have pressed for years to drop the distinctions (at least when talking to patients) I would agree.
Despite all of that the question I would ask (and yes I have been diagnosed with SS) what possible difference does it make? Being able to say I am "primary SS Type 2 would not change your treatment as they treat based on current circumstances. There is not an autoimmune disease out there people "get" so it won't help there. SS for most patients is not a big deal (sorry but its true) so that wouldn't change folks (or even many practitioners) perceptions either. Advanced Sjogrens is now frequently defined as what is happening including the new (and more commonoy used term "“primary-progressive MS” which is different from the common “relapsing-remitting” MS. and is marked by a more gradual period of deterioration, reflecting inflammation and damage in the spine (i.e. progressive myelitis). The only way to know the difference is if the diseases responds to a trial of immunosuppressant medication which may be helpful if progressive myelitis is occurring because of Sjögren’s syndrome.
The important issue and really the only issue is NOT terminology which varies widley with the Syndromes as they are collections of symptoms not well defined disease, but rather if you are getting treatment. If terminology is stopping you from treatment, then there is a problem. Equally important IMO is coping on a day to day basis. For that you look to your peers.
I think its a starting place. We are all so different in when and how we were diagnosed and how we are affected, but there are similarities in all of us. I don't think there will ever be a textbook anything when it comes to us, I drive my doctors crazy because I don't make sense. Thankfully, they don't think maybe m crazy, they believe something is wrong, but it frustrates them because they don't always know where to start to help. But they listen to me. We talk and we try to figure it out. So far the thing that has helped me is that they listen and have learned what is normal for me. I'm not sure about stages, at least for me, but I think its a good idea for helping doctors learn to understand us