When I was diagnosed with primary Sjogren’s Syndrome by Dr A-, some 12 years after I first became ill in 1990, I was relieved to have a name for the invisible illness that had wracked my life with its silent misery. However, there was one part of the diagnosis that bugged me: ‘working diagnosis’. It made it seem somehow less real. When I saw Dr A- for the last time, some 7 years later on the eve of his retirement, he reaffirmed his opinion that he had got the diagnosis right. He was respected in his field and at the end of a long and illustrious career.
However, my diagnosis was made without one of the key parts of the formal diagnosis: a positive finding of antibodies to one or both of the Ro (SSA) and La (SSB) antigens. This means my Sjogren’s diagnosis was made although I was seronegative (in other words, a key blood test didn’t prove it). Not everyone with Sjogren’s has antibodies to SSA and SSB, but it still bugged me that I didn’t have the gold standard of ‘proof’ of my illness.
Recently, some 11 years after that first test for SSA and SSB antibodies, I had the test repeated, understanding that the results can change over time. I’m still seronegative.
I also recently attended a fine webinar on Sjogren’s syndrome given by Dr Arthur Bookman, hosted by the Sjogren’s Society of Canada. The focus of the webinar was the formal diagnosis of Sjogren’s. I later came to learn that some rheumatologists believe that those without the SSA and SSB antibodies or other key tests such as positive anti-nuclear antibodies (ANA) do not have Sjogren’s. This may explain why, when looking for a replacement rheumatologist, the doctor asked if I had positive ANA – I didn’t – then asked if I took antidepressants – I did – and said I probably didn’t have Sjogren’s. Seeing as Prozac was meant to replace a generation of anticholinergic antidepressants (meds that were strongly associated with such symptoms as dry mouth), I don’t know what his slant was, or whether he was just prejudiced against depressed people! Needless to say, I didn’t hire this guy.
Regardless, I have nearly all symptoms of the disease, but I don’t have the standard of diagnosis that others have, including those who found out incidentally about their seropositive status, while showing few of the symptoms.
The diagnosis of Sjogren’s, whether primary or secondary to another autoimmune disease, is set out according to various combinations of diagnostic criteria, but four of the most objective tests for Sjogren’s are:
– reduced tear production shown by a positive Schirmer’s test or Rose Bengal staining test. The Schirmer’s test measures the flow of tears by their absorption by a small piece of litmus paper. People with Sjogren’s typically show tear production of less than 5 mm in 5 minutes. I had 0 mm in 5 minutes.
– positive lip biopsy: This test shows inflammatory damage in the salivary gland. I don’t know whether it’s the expense of the test, or my doctor’s confidence in the diagnosis, but it was never suggested that I have this done.
– reduced salivary flow: This can be done in a simple way (such as spitting into a cup) or using fairly complicated scanning technology. An unstimulated whole (all salivary glands) flow of less than 1.5 ml in 15 minutes is considered positive for Sjogren’s. I had 2 ml in 15 minutes.
– positive SSA and SSB antibodies. I am seronegative.
Which means that after 23 years, I’m still wondering a little about my diagnosis. As Dr Bookman expressed, most treatments are ineffective for Sjogren’s (as was my experience), and the quality of life of Sjogren’s patients with the systemic form of the disease is also greatly impacted (also my experience). My present internist, told me that many autoimmune diseases rely on meeting several often subjective conditions, and that diagnosis will one day be clinched by genetic testing that will lead to an altogether different classification of autoimmune disease, and perhaps new treatment modalities. I can only hope.
On one hand, it’s quite possible that my first doctor was right and I do have Sjogren’s syndrome, regardless of what some doctors may think. Even if my illness is one day diagnosed as something else as more symptoms emerge over time (and they do), or I’m diagnosed as having Sjogren’s secondary to something else, my efforts at advocating for this disease, particularly the systemic form, are not in vain. Many people are suffering, and few are listening. I hope to change that.
In a future post, I’m going to discuss my symptoms and the most significant test results I’ve had. I’m also going to talk more about the quality of life issues raised by Dr Bookman in his talk. I’m hoping this will be useful both to other people with Sjogren’s, especially those who are seronegative, and for general education to show that Sjogren’s syndrome is so much more than ‘dry eyes and dry mouth’.
Blessings,
Jane
16 Responses
AMAZING post Jane! Thank you so very much for all the useful information. Becasue I am seronegative, my Rheumy won't further test me for Sjogren's. He feels all the "issues" are due to the meds I take for another autoimmune. I've pointed out repeatedly that I've dealt with these problems so much longer than that (first diagnosis for dry eyes was when I was in 5th grade!)
Now I'm armed with more knowlege for my next visit. Thanks again!
Hi Chris!
I'm so glad it helps you, hon! *hugs* I know that the second rheumy I saw – the one I didn't bother going on a second date with – seemed to believe it was related to drugs, but like you, all the symptoms persisted during those times I wasn't on any medication. I think they like to hold on to some convenient truths that make their diagnostic lives easier, but when it's at the expense of the patient, I think it borders on malpractice. I know that I can't expect to get any treatment that works (or even that I can tolerate), unless there is some massive shake-up in understanding of the mechanisms of autoimmune disease, but I would like to stop all this diagnostic screwing around. I believe I have Sjogren's, but in some rheumatologist's books, I don't. I can't help but think if they had the right test (like the genetic testing my internist promised would come one day), it would be settled once and for all.
People say things like, "Aww, you should be relieved you don't have a label," but no, there's no relief when you're suffering all the time from some unnamed malady. I want and deserve answers, damn it! :)
In my next post I'm going to include all the significant results from all my tests over the years. I'm hoping that even us seronegative folks will find commonalities and at least find that there is some method to this Sjogren's madness.
Love and hugs,
Jane
Sjogren's Syndrome does have typical symptoms of dry eyes & mouth, but for many of us like you Jane & I, it goes way beyond that. Mine is also primary as I don't have RA, I do have Osteoarthritis & Fibromyalgia along with it, but that is just 3 of many chronic health issues I have. Life is far from typical isn't it? no two days are alike. At least life isn't boring eh? lol. I feel so blessed to have gotten to know you and Carmen. I know like me you put on a brave face and get through one day at a time, not knowing what symptoms of what condition will rear its ugly head today. We need to see much more research and knowledge out there about it. I know every time I look up SS its the exact same small bit of info. I love your blogs, and it was perfect timing. Because of having to move, I have been so stressed, which has me in a major flair up right now as well as increased anxiety attacks. I went to see my Doc the other day and got my ativan strength increased a bit, just until the move is over with. So this blog has come at the right time. xx
Love & Light
Joan Bloch
Aww, hon, I hear you on all this – the stress, trying to put on the brave face, making it one day at a time. Yes, yes, yes! I am so messed up in a flare at the moment. I am sleeping at all weird times of the day (not enough at night), and just so tired like I've been hit by a truck. I agree that research and knowledge is so needed. There is so little known about this illness, and honestly it's hard to compete with some of the fundraising juggernauts associated with other conditions. I don't begrudge them their needs, but the problem with so many of the autoimmune diseases is that we're all too wiped and in too much pain to do the fundraising. I barely leave the house, so how the heck could I organize a walk, or whatever it is that is trendy in fundraising these days?
I too have fibromyalgia, and it frustrates the picture. I've had this pain in my right kidney area for 2 years now. They press on my body and say, well, it's just superficial. They don't get they could press anywhere and get the same reaction. This pain is deep. I don't want to wake up one day and discover I've got renal failure on top of the other things. Lately they've started saying to me, well, you're just getting older. This started happening to me 23 years ago, when I was 24 years old! Don't tell me I'm suffering from old age. LOL
I hope you can take care of yourself during this move, hon. I know how arduous it is. In my first 28 years, I must have moved some 20 or so times. I'm glad that it's only happening every five years or so now! Hang in there, and rest as much as you can. Write whenever you need to vent.
Love and huggles,
Jane
Hello Jane :)
As you know, I have fibromyalgia. I also have ME, TN… ohh.. and other things lol. I don't remember the names atm.. fibro fog.
I just wanted to let you know that when my immune functions have been tested, they have always come back normal. Now.. I have been diagnosed with all these illness', and apparently they are 'linked' to auto immune deficiency. Hmm… well I don't 'fit' lol. Nothing new in my life tho!
I think its all over the place… these 'tests' to 'diagnose properly'. Honestly, I don't think anyone really knows what it 'should be'. It's very distressing for all of us out here suffering with pain of all kinds every day. Would be nice if they just said the truth and let us be taken seriously instead. Like, 'we don't really have a valid test… we are just going with what it is we have found so far' kind of thing.
Gosh.. so many people are suffering from these Invisible Illness'.
Shakes head.
Some days I just loose faith in people. The lack of care and understanding in our medical professionals cough… astounds me.
But.. the Taurean steam inevitably comes out, and I stand tall with my head held high again.
Keep up this Great Space Jane! Love the sharing xoxox
Hi Tan!
Thanks for writing. I think one thing that has really come to annoy me (more so in recent years) is that despite the inclusion of 'normal' ranges on blood and other tests, the doctor will ignore any outlying results and just say 'normal'. It's only that I have been active in asking for all my results over the years (damn it, they're MY test results!) that I find often they will say something is normal when it's not, consistently. Now maybe I'm a consistent outlier, but on so many different tests?
About two years ago I started developing a pain deep in my right side. Doctors will examine the area and because of my FM, I will flinch, they don't get that the pain is below the 'regular' surface pain. I've had scans, and they don't find anything. Now I know systemic Sjogren's can involve the kidney, so I was worried. My results are showing an increasing amount of blood and protein in my urine, consistent with a kidney problem. It's in an 'abnormal' range. But the internist says, your results would need to be ten times worse before we would do anything. Hello? I need to have renal failure before you would act? Ack. Others say, well, you're getting older. I guess that's why I don't bother seeing doctors at the moment.
Thanks for the encouragement, hon!
Blessings,
Jane
I have a very "loose" diagnosis but my Sjogrens is considered secondary. When I asked the doctor about it he said a great many of his patients had Sjogren's and if I wanted to see a lot of doctors he could refer me to the Clinic we have here in Toronto that Dr Bookman runs.
I said no so that I would not sound needy, and have been lucky to find good doctors to treat the symptoms – but primary Sjogrens,, despite that I hear they're saying that it is all one disease, seems a lot more complex than dealing with dryness.
I have had the Schirmers test, and the staining, and also punctal plugs and Restasis. No biopsy and no blood tests for anti Ro and La
And are you moving too? – what a lot of stress that must be.
Annette
I would so love to attend the Sjogren’s clinic there. Money and energy to travel are in short supply, besides, they might not want to see an out-of-province patient. I can’t help but think I would get so much more helpful guidance there. Or maybe I’m just like Dorothy, and the answer is in Kansas, not in Oz! :)
Your Sjogren’s is secondary simply because it is secondary to another connective tissue disease, RA. As this FAQ on the SS Foundation site reminds us (https://www.sjogrens.org/home/about-sjogrens/sjoegrens-faqs), the classification into primary or secondary has nothing to do with how severe your disease can be. You can be either and 'just' have the classic dry eyes and mouth, or you could have full systemic involvement.
As you say, and as Dr Bookman said in his talk, there are not really many effective treatments for the disease – you are just easing the symptoms.
Moving – no, not me, luckily. Joan (further up in the comments) is moving. I've moved way too often, and I'm quite glad if I can have a chance to gather some moss while sitting here. :)
Blessings, my friend.
Jane
Gathering moss my foot. Have you all got your colourful wardrobes for coming out of winter? I am wearing my notorious purple fleece pants and shocking pink and aqua (so 50s). One item is motheaten. So comfortable. Moths love me.
I actually saw Dr Bookman this week (as in glimpsed in a hallway). I joined Patient Partners and we were doing a demo so was at western Hospital
I wish you could come for the patient conference in May but it's a long and expensive trip for a one day conference, and we can't video the conference to spread it around. I am going and will try to do some live tweeting and take notes.
LOL, my summer wardrobe is about as colourful as my winter one, which is quite good. Growing up, my mother favoured my wearing lots of pastel colours and I went along with it. Eventually when I started buying my own clothes there were lots of rich colours, purples, greens and even some orange. :) Our daughter, K-, has got an amazing summer wardrobe. I'm looking forward to seeing it again. It sounds like you will be making a statement once you step out of the snow and ice!
I'm so glad you're going to the conference. I'm hoping that many people there will be able to tweet and blog – so important that the message gets out to all the people who can't go. Summaries after the event are great – I think live tweeting would be quite stressful – I don't know my brain could hold it all together LOL. I love some of the round-ups that Julia at Reasonably Well does. Makes you feel like you were there! :)
Take care!
I am not a quick typist, especially on a tablet computer so I'll try. Maybe we can get a great patient advocate person to come and live tweet it specially. It is not a very expensive conference to attend and would be so worth it
Annette, do you happen to know who is hosting the conference? I wonder what they have in terms of media relations, facilities, etc. Of course, like most things to do with Sjogren's, there is probably little funding. :(
The conference is run by Sjogren's Canada and they try to keep the fees low so patients can attend easily. I'll ask Lee about the possibilities. Their website is http://sjogrenscanada.org/
Thank you. Of course, I don't want to create any work for anyone. Putting on a conference is a huge stress. I know I couldn't do it anymore. Perhaps like their recent webinar, Sjogren's Canada could later make available selected information like speaker's talks for a fee.
I don't think it is a matter of cost so much as that the material belongs to the speakers so they do not give the society the rights to tape it and use it. They are quite good at finding interesting speakers. They had Dr Yepes last time who knows a lot about dry mouth. I was interested to find out that though salagen does stimulate saliva flow it actually does not make a difference to how the glands do over time.
I think I'll do a blog post about Sjogrens Canada and the last conference. All I need is to find my speakers book. Maybe I can wangle an extra book for you if there are leftovers
That makes sense, re: protecting the speaker's material. I will just listen with interest to reports about the conference. Thanks, Annette!