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’.