I feel so tired and sub-par all the time that I wonder how I would know if there
was something else wrong. So many things can be attributed to Sjogren's that it
seems as though it might be too easy to assume everything is.

In particular I'm wondering about lymphoma. How would you catch it early?

Thanks.

A:  For Lymphoma I will check a beta2 microglobulin, LDH, and IEP (immunoelectropheresis, for monoclonal antibodies) every year or so. These have not been validated in clinical studies to be predictive or cost effective...maybe those types of studies will be done in the future. Beta2microglobulin is beginning to be used as a "biomarker" in recent clinical trials on Sjs, so we may get additional clues on how helpful it is. The older literature has supported such markers as cryoglobulins, decreased complements, or positive serologies (ANA, SSA/B etc) that then become negative (without a clinical remission) - as predictors of lymphoma development in Sjs. Cryo's in particular are very Lab variable and hard to collect...often not very practical in the office setting.

There are no Sjs specific tests/studies that are currently recommended for the Primary Care provider to do. The usual would cover most things. Since, thyroiditis commonly occurs with Sjs, and can burn out to become low thyroid (hypothyroid), and especially in setting of Fatigue. I would suggest a TSH (thyroid stimulating hormone), a CBC with diff that is usually done would pick up signs of anemia, which can also cause fatigue.

Being familiar with ALL the possible things that can happen (but may never) is important to really answer your question. Maybe your PCP would appreciate a copy of the Sjogren's Handbook, recently updated by the Sjogren's Foundation (www.sjogrens.org) or asking if their office will sign up for the Sjogren's Quarterly for healthcare providers, also through the Sjogren's Foundation. That publication is FREE to healthcare providers and has excellent clinical and research articles that help keep us all up-to-date.    **Disclaimer: I am biased in thinking these are good resources, as I currently serve as a Board Member on the Sjogren's Foundation, and on the Editorial Staff of the Quarterly. K Hammit, VP of Research for the SSF is the Editor. Hopefully, this is a good "bias".

dr c

A:  Could be. From the dr's perspective, it's in the "differential diagnosis". In my experience, the multi-system illnesses that go on for 6 mo or more are either: chronic infection, autoimmune, or paraneoplastic. In 2 yrs with investigation and testing, usually infection and underlying malignacy have been ruled out, as best as possible.

The weight loss while still eating, with normal thyroid function, is usually malabsorption. This can occur in wheat allergy/Celiac and other autoimmune disorders.

dr c

A:  The bolding above is mine.

I encountered similar comments and experiences from patient's in our office this week...and most weeks.... I don't really have an answer. I thought that was part of being a physician. The patient presents because they have a problem, the physician listens/asks questions/creates a hypothesis and plan to test it/collects the data/sees if it fits/recommends a plan/pt reports they are better OR not/rework the plan if needed/refer the pt as appropriate. This is not a process I have EVER been able to do in 5-30 min, which is why patient's get backed-up, like airplanes trying to land, most days.

I suspect the answer is multi-factorial, and the patient, if they can take a deep breath and relax (good luck when you're loosing your health, job, insurance...) can come up with a pretty accurate list of probable reasons.

Maybe the Collective Voices of Patients and those trying to get educated about healthcare issues need to turn the VOLUME up, take their business elsewhere, complain to their human resources person/insurer... Those affected by Breast cancer and HIV have been much more effective about being heard, getting awareness and resources.....

dr c

A:  To make a diagnosis of Sarcoid, one would really need a biopsy of the hilar node showing "non-caseating granuloma" - the hallmark of Sarcoid. Given, the complexity of the story....to make a diagnosis of Sjogren's, one would be served to obtain a labial (lip) salivary gland biopsy that showed "focal lymphocytic sialadenitis". If the Focus Score was greater than 1, this would provide even stronger evidence of a lymphocytic process.  However, even IF a diagnosis of Sjogren's was made.....the STORY seems bigger than Sjogren's alone could explain with any degree of confidence....

If one goes back to the first unusual finding...the "benign" lymphnode...could that be a clue that a virus like Mono/EBV was present..driving lymphocyte proliferation????

Could the then rash, peeling eyelids with autoantibodies have been the autoimmune condition Dermatomyositis ??? This can be primarily autoimmune or an autoimmune response to Cancer. Not a commonly reported association with cervical cancer...but with a cancer aggressive enough that was treated very aggressively.....it seems possible that the host immune response would be trying to contain it. If there is this connection, then when the cancer resolves....the autoimmune piece down regulates...

Autoantibodies are just immune reactions to self tissue...they do not just by themselves make a diagnosis of an autoimmune disease.

A separate response to WHY will no one Listen will follow?

dr c

A:  Anxiety, Depression, the Fibro diagnosis....then Hashimoto's thyroiditis (autoimmune), then Sjogren's (autoimmune). Fibromyalgia is a diagnosis of exclusion. First, all other causes of pain, fatigue etc are ruled out. All blood work is normal. No autoantibodies, no inflammatory markers and a history and physical exam consistent with Fibro. Sometimes, the Fibro diagnosis comes when there is no other explanation for the patient's symptoms...but then STAYS, even when a diagnosis of another condition, like an autoimmune disease, is made.

Hashimoto's for example can be associated with tendonitis and a positive ANA. Sjogren's patient's can have an inflammatory arthritis. The Sjogren's Foundation's  recent edition of their Handbook has a Chapter on Arthritis in Sjogren's (www.sjogrens.org). Perhaps reviewing that with your Rheum and exploring other options you might try to see if their are other causes for your symptoms, and other options to improve the quality of one's life. Water therapy or a jump start with a Physical Therapist can help arthritis pain without ongoing injury.

dr c

A:  Autoimmune diseases are included in the differential diagnosis of MGUS. It might also be possible that the low Immunoglobulins predated the MGUS and contributed to increase susceptibility to infections. Furthermore, infections/inflammation can drive the immune system into overdrive...and favor a monoclonal process verses a more "normal" polyclonal process.

So....Sjs could present this way. With a good history, and possibly a few tests your physicians should be able to rule this in or out for you.

dr c

A:  Lot's of medications are associated with dryness. Major ones are anti-depressants, pain medications (opioids), and diuretics. I did not find a central list of ALL med's associated with dryness.

The best way is to search a specific medication and dryness and/or read through all the side effects associated with a specific drug.

If symptoms started after starting a new medication, then the possibility that it may be contributing is high. Only way to know for sure in an individual case is to stop the medication and see if the dryness improves....but this should be carefully discussed with your treating physician...regarding the risks/downsides of doing so.

There is a Product Directory on the Sjogren's Fundation website..but it is directed at products that are recommended to be used in Sjogren's.   www.sjogrens.org

dr c

Though I have not been formally diagnosed with Sjs, I have many of the symptoms
that I read about in your book, A Body Out of Balance.  I have been diagnoses
with dry eyes.  I also suffer from sore joints, burning mouth and tongue, tender
neck (glands), vision problems," cracking" joints especially in the AM.  Two
years ago I was tested but the ANA results were negative. I was tested again
last year and the ANA results were negative.  I am VERY frustrated.  These
problems arose after going through menopause at age 49.  I am now 56.

Can you tell me of a reputable doctor in my area?  I live north of Vancouver,
WA, near Portland, OR.

Thank you so much!

A:  Frustration for 7 yrs can't be good for health!

~40% of pts with a diagnosis of Sjs by lip bx have negative SSA/SSB.  Sometimes the ANA is negative  but the SSA and/or B will be positive or there may be a positive RF. So Sjs has not been ruled out yet.

Menopause can lead to dryness symptoms etc, but what you are describing seems a bit much just for that. Hopefully your Gyn has weighed in on this point.

The National Sjogren's Foundation or a Local Sjogren's Support Group may be able to provide a specific name for you in your area.

dr c

This happened last month and my chest was sore when coughing so I went to my GP.
She also took a regular blood test to check on my meds' effects. My white blood
cell count was low so she asked me to come off Immuran for a week while I was
taking an antibiotic and see if I improved.

I did that and all worked well and went back on Immuran again, I also take
Plaquenil.
Yesterday while sitting down at work I felt a pain in my right foot. It got
worse and I felt shivery and my hands were very cold. When I stood up to leave I
couldn't walk properly and limped to my car. The pain was vey bad even when my
foot was motionless. I took pain killers and it passed very soon. I was very
tired for the rest of the evening.
I googled it and found this forum and the following question:
(Q:  I was diagnosed with Sjogrens Syndrome 30 years ago. Apart from the usual
symptoms, dry eyes, dry mouth, skin rashes, flare ups of Oral Lichen Planus I
occasionally suffer from severe pain in both feet.  Pain starts gradually and
builds up over following 6 minutes when it becomes excruciating. If i can apply
cold compress as soon as the pain begins this can sometimes help.  Pain is on
sides of feet between little toe bone and ankle bone. I have asked my GP and
Rheumatologist but they cannot give me an explanation.  Are you familiar with
these symptoms in Sjogrens patients? I hope you can throw some light on this
mysterious pain.)

It describes my pain and location on foot precisely.
Do you think this might have occured because I came off the Immuran for a week?

I am writing to you because I live in Ireland (population 4 million) and there
is not much experience of the disease here, though I am getting good care.

A:  A severe pain that comes on suddenly without an obvious insult...raises the question...could this be a process affecting the nerve and/or blood vessel to a specific location. Good news..it resolved, but thus unlikely that the cause will be determined with any certainty.

Some with Sjs have more evidence of immune-complex activity (more antibodies reacting to target antigens). These immune-complexes can cause inflammation. This could be directed directly to nerve tissue or blood vessel tissue, or even to the small blood vessels that feed the nerves (vaso vasorum). The later can lead to a specific type of vasculitis called Mononeuritis Multiplex, and this is known to occur in Sjs, but is rare.

Immune modulation can decrease inflammation caused by immune-complexes....so it could be theoretically possible that a break from taking Imuran might be a co-factor in....whatever really did occur...

dr c

A:  Fortunately there is increasing attention and resources to help those affected...web sites...books...support groups...supportive family and co-workers.  Bottom line is how could Anyone Cope....What are the options...Most choose to cope the best they can, with the resources they are able to access.

From this doctor's perspective, I don't know how you can move forward until you have a diagnosis or at least a hypothesis of what is "out of balance" so you can design a treatment plan and see what works and what does not work. Until then, one is operating in the dark.

Hope the "luck" continues!

dr c