This article describes the clinical and diagnostic differences
between primary and secondary Sjogren's syndrome.
Sjogren's disease is a chronic autoimmune disease that occurs as a localized syndrome primarily causing mouth and eye dryness (sicca syndrome) or as a systemic disease affecting multiple organs. Sjogren's is suspected of affecting about 2-3 million Americans, 90 percent of them women. Frequently, the condition remains unrecognized and untreated, and when treated, the average diagnosis is reported to take 3.5 years. Women in their fourth decade of life are most likely to be affected. The complaint most often listed at the time of diagnosis is mouth dryness. In Sjogren's syndrome, white blood cells known as lymphocytes invade the exocrine glands. The exocrine glands produce needed moisture for lubricating and bathing the body's organs.
Sjogren's syndrome occurs as a primary or secondary disorder. As a primary disorder, a patient with no known connective tissue disease or arthritic symptoms develops dryness of the mouth, which is called xerostomia, or dryness of the eyes, which is called xerophthalmia. Eye dryness caused ocular discomfort, and the eye may adhere to the eyelid causing conjunctival or corneal surface injuries and keratitis. Eye symptoms are related both to eye gland destruction and a diminished response to nerve impulses. Other organs, including the digestive organs and vagina, may also be affected. In secondary Sjogren's syndrome, patients with another autoimmune disorder such as rheumatoid arthritis, develop mouth and eye dryness.
Up to 25 percent of patients with rheumatoid arthritis later develop secondary Sjogren's syndrome. Other autoimmune disorders in which Sjogren's occurs include: systemic lupus erythematosus, scleroderma, mixed connective tissue disease, relapsing polychronditis, and polymyositis. Patients with Graves' disease treated with radioiodine may experience damage to salivary or parotid glands causing symptoms similar to those of xerostomia. Patients with primary Sjogren's are at risk for later developing other autoimmune conditions, including Hashimoto's thyroiditis, and certain malignancies such as pseudo-lymphoma, a proliferation of lymphocytic white blood cells that occurs in about 10 percent of patients with primary lymphoma. Up to 10 percent of these patients with pseudo-lymphoma or 1 percent of patients with primary Sjogren's may develop non-Hodgkin's lymphoma.
DIFFERENTIAL DIAGNOSIS
Sjogren's syndrome must also be differentiated from drug-related xerostomia, which can be caused by up to 400 different medications. Drugs known to cause sicca syndrome include benzodiazepines, tricyclic antidepressants, and antihistamines. External beam radiation and radiodine treatment, especially in the higher doses, used for cancer, may permanently damage the salivary and lacrimal glands. Mouth breathing due to nasal obstruction may also cause similar symptoms. Patients with acidosis, hepatitis, fibromyalgia, chronic or sclerosing sialadenitis (inflammation of salivary glands) and patients with human immunodeficiency virus infection with salivary diffuse infiltrative lymphocytosis syndrome (DILS) also have symptoms and some laboratory test results similar to patients with Sjogren's syndrome. These patients who have diseases with symptoms mimicking those of Sjogren's syndrome will not have positive SS-A or SS-B antibody tests.
DIAGNOSTIC CRITERIA
Four criteria are used to diagnose Sjogren's syndrome: objective documentation of dry mouth or dry eye, positive salivary gland lip biopsy results, an elevated ANA titer, primarily due to a positive SS-A antibody test. Overall, 70-90 percent of patient's with Sjogren's have a positive ANA test, and 40-60 percent have SS-A or Ro antibodies. SSA-B or La antibodies are seen in 20-40 percent of patients, and rheumatoid factor (RF) is seen in 60-90 percent of patients. In general, men with Sjogren's are less likely to have elevated autoantibody titers. The C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) test results are usually elevated. Blood immunoglobulin levels are also usually elevated (hypergammaglobulinemia) in Sjogren's. MRI or ultrasound examinations of the parotid gland are also used to evaluate glandular insufficiency. Sialometry is used either with or without stimulation to evaluate deficiencies in saliva production. Both parotid and submandibular flow may be measured.
The Schirmer test is often used to measure the volume of tear production.
EXTRAGLANDULAR SYMPTOMS
While most patients with Sjogren's syndrome have symptoms limited to sicca syndrome, a subset of patients may develop extraglandular symptoms, that is, symptoms not related to the glands. These symptoms include fatigue, rash, cutaneous vasculitis, Raynaud's phenomenon, arthralgia (joint pain), myalgia (muscle pain), myositis (muscle inflammation), frank arthritis, and a particular form of erosive or aggressive osteoarthritis affecting the hands and feet. Internal organ involvement includes: lung inflammation (pneumonitis), liver disease, nervous system inflammation (neuropathy), kidney disease (glomerulonephritis), bruising related to elevated immunoglobulins; leukopenia (decreased white blood cell count); thrombocytopenia (decreased platelet count); and anemia. There is also an association with head and neck lymphoma. Gynecological complaints include vaginal dryness, diminished urine, and dyspareunia (urine leakage).
PRIMARY VS SECONDARY SJOGREN'S
Patients with primary Sjogren's disease are more likely to have both SS-A (Ro) and SS-B (La) antibodies or SS-A antibodies alone. Patients with secondary Sjogren's are less likely to have these antibodies. In Sjogren's test for SS-A measuring both 52 and 60 kD are positive, whereas procedures that measure 60 kD or 52 kD antibodies alone are negative.
The copyright of the article Sjogren's Subtypes in Rheumatism is owned by Elaine Moore. Permission to republish Sjogren's Subtypes in print or online must be granted by the author in writing.
I have had symptoms of Primary Sjogrens since I was a child, although the
sicca symptoms were not as annoying as the rheumatic. After diagnosing
myself I had it confirmed by a rheumatologist several years ago. Well, at
least I have a diagnosis, but often wonder whether it's worth having all
these doctors and pills. One of the most emotionally draining problems I
have is convincing my doctors that I have an inflammatory response from
medical radiation. My radiation therapy was aborted l4 years ago due to my
sensitive skin as well as the mastectomy incision opening. An angiogram,
cat scan and pre-op chest x-rays for a hysterectomy had me almost disabled.
Everything I did was a monumental chore, so had to try Diclofenac as
steroids make my spine worse. At this point I can honestly say that cancer
does not frighten me as much as the thought of the pain I have following
any type of radiation tests. I have decided to do the homeopathic route
although I have to say it is hard on my budget.
Jan 29, 2009 8:16 AM
Guest :
I have been diagnosed with primary Sjogrens after eight years of severe
symptoms, the worst including extreme fatigue, muscle weakness, shortness
of breath without exertion, body-wide pain and constant total lack of any
energy. The dry mouth and dry eyes are bothersome, have had since
teenager, but I have to say, they are the least worrisome to me compared to
the other symptoms. My symptoms listed above have become increasingly
severe, to the point that I am afraid that if I don't get help to stop the
progression, I will be unable to have any function. My doctors don't seem
to get the degree of urgency I feel. I have tried so many meds, none seem
to help with pain or the fatigue and only seem to cause more symptoms and
conditions (stomach ulcer) to deal with as side effects. I totally don't
know where to turn or what to do but I feel my health dwindling quickly and
I'm very afraid. I had to stop working in June on the advice of one of my
physicians. Work is totally out of the question. Just maintaining myself
to make it through a normal day is a chore and sometimes unattainable. I'm
scared. Any advice?
Jan 29, 2009 11:32 AM
Elaine Moore :
Hi, I'm sorry to hear that you're not feeling well and that you're not
getting the help you need. You might consider changing doctors,
finding a rheumatologist with a specialty in systemic Sjogren's or even a
naturopath or integrationist physician, who understands about treating the
whole body. With changes in the American with Disabilities Act that
came into effect this month, you could be eligible for disability if you're
still employed an are on leave. As part of this act, several disability
claims that were denied before have been reversed. You might also
want to do some research on low dose naltrexone, which has been used
successfully off-label in patients with Sjogren's. Best to you, Elaine
Feb 19, 2009 11:44 AM
Guest :
I came across your website today and saw I had posted a comment on Aug. 2l,
2008 regarding my inflammatory response following medical radiation in any
way, shape or form. Just want to say that my neurologist finally diagnosed
my mastectomy and lymph node pain as a radiation injury to the brachial
plexus which involves the ulnar nerves. Out of all my M.D.'s my G.P. was
the most difficult to convince. How exhausting! I just want all of you to
know that some of my sensory neuropathy problems have improved with the
homeopathic meds Ziquin "Full Spectrum Fusion" as well as my
gastro problems with Ziquin "All Xymatic Relief ". The
Diclofenac continues to work well for rheumatic pain. I do wish they would
consider looking at integrative medicine. This Sjogren thing can be
devastating and we need all the help we can get.
Feb 24, 2009 2:57 AM
Guest :
I am 51 years old. I have had symptoms like numbness and pins and needles
in my arms and legs for 18 years. Lately I have 1/160 or 1/320 ANA and
sometimew low C3 (border line). Normal CRP, Ra , negative SSA, SSB
excellent ESR I experience some arthritis problems and some minor dry
eyes and mouth. My doctor put my on plaquenil and told me not to worry. What should I do?
Feb 24, 2009 10:50 AM
Elaine Moore :
Hi, Plaquenil is used to reduce symptoms in many different autoimmune
rheumatological disorders. If you're seeing a rheumatologist you probably
are best off following his advice and perhaps focusing on a diet that
reduces inflammation. This involves avoiding sugar, saturated fats, cheese,
red meats and other foods that promote inflammation and adding more foods,
such as fruits and vegetables, that are high in antioxidants and help
reduce inflammation. Many people with autoimmune disorders notice
improvement with dietary changes. Best, elaine
Mar 6, 2009 7:13 PM
Guest :
I have had very dry eyes for several years now that preclude me from
wearing contacts. I've had the punctum plugs placed which didn't help much.
Around 5 yrs ago I was diagnosed with Hashimotos thyroid disease and had an
ANA of 1:1280, homogeneous. Dr said it was from the thyroid. Lots of
fatigue. I finally felt better. After my TSH was under control. Now I'm
still hving severe dry eyes and also have dry mouth (feels like food gets
stuck at back of my throat), severe fatigue and dry cough. Knee pain too,
but not sure if it's related as I've had a couple of falls and arthriscopic
knee surgery for torn meniscus 8 months ago. Now having scary memory
problems that took me back to the dr. My ANA is1:320 homogeneous and 1:320
speckled. Antibody specific tests are negative. My dr. is referring me to a
Rheumatologist. Does this sound like Sjogrens or SLE?
Mar 6, 2009 9:17 PM
Elaine Moore :
Hi, With the symptoms you're mentioning it sounds more like Sjogren's
although your doctor will need to run more tests to diagnose your
condition. Sometimes, early on symptoms will wax and wane and lab results
will vary. Some disorders can always seem on the edge of turning into
full-bloom disorders and sometimes they tend to resolve with periods of
symptoms alternating with periods of remission. For these reasons diagnosis
isn't always clear-cut. Best to you, Elaine
Mar 9, 2009 12:55 PM
Guest :
In the seem to be never ending search I came across your site. I have had
increasing difficulty over the past few years with muscle & joint pain.
I have over the years tested postive, then negative for Lyme, Lupus and I
keep testing positive for Sjogrens. My Dr. has order a salivary glan biopsy
but I cannot seem to find someone to do this who is covered under
insurance, otherwise $600 out of pocket (which is begining to seem
inevitable). I've been told I also have the wonderful clinical diagnosis
of fybromyalgia. The worst part seems to be the increasing pain mostly in
both feet, I can't stand all day or even part of the day anymore. I was
always outgoing and feel like such a wuss to say the least. So what is my
question? Have you heard of Sjogrens causing extreme foot pain? I also
feel as though I did a hard core workout when I haven't! I wish I felt up
to a hard core workout. I've gained weight, that's not helping either. I
just really want to get to the bottom of this and figure out a way to feel
like myself again. My husband can't even massage me, it hurts with minimal
pressure. I have also had neck and lumbar denervation due to pain, and
am now considering a breast reduction! My Dr. said he would be supportive
and write the necessary letter, anything to releive the pain. It used to
be pretty limited to neck and back, but now both feet and wrists, this is
crazy. My mother is rhumetoid and boy do I now give her so much
credit, I never knew how bad she has had it all these years. Signed....the wuss
Mar 9, 2009 2:19 PM
Elaine Moore :
Hi Wuss, Is there any way you could have plantar fasciitis? That can
occur from even walking in the wrong shoes. The last time I had it it took
months to go away. I got it from doing a very easy workout in my regular
work shoes. I've known people who've gotten it from dancing or even walking
barefoot. Do a search on it and see what you think. PF is also more common
in hypothyroidism, and hypothyroidism can cause symptoms of arthritis. I'm not sure how insurance covers the diagnostic tests for Sjogren's. You
might want to look at the diagnostic criteria listed at the American
Rheumatological Association and see if you have enough evidence for a
diagnosis without the salivary test. Since SS affects the glands that
produce moisture I'm not sure about it affecting your feet. The arthritic
component of Sjogren's probably can affect your feet and your doctor could
run imaging tests to look for degenerative changes. I hope you're
able to figure this out and find relief soon. Best, Elaine
Mar 17, 2009 6:58 PM
Guest :
Ok i hope this is a good site. My 12 year old has sjogrens and yes positive
ssa anti ro high levels of iGg and the whole nine yards. She has recently
been cnstantly loosing her voice for now apparent reason. she does sing in
a honor choir but from day to night it can change. She suffers so bad from
fatigue and mmody well thats putting it lightly. She has had a clear runny
nose and o voice for a while now. I am severly worried about it manifesting
into cancer especially at this young age. What am i to think. she has it so
young i wonder what her future holds. (mom of four looking for advice
anyone have any suggestions.)She is currently on Methotrexate, plaquinal
folic acid, Exc. Thank You; Julie (Miranda's Mom)
Mar 18, 2009 8:26 AM
Elaine Moore :
Hi Mom of Four,
Some of the symptoms you mention sound similar
to those seen in hypothyroidism. Thyroid disorders are fairly common in
Sjogren's patients. People with autoimmune diseases are also more likely to
have seasonal allergies. Why don't you call your doctor's office and report
Julie's symptoms. They can always order outpatient lab tests without your
having to have another appointment. Best, Elaine
May 26, 2009 11:36 AM
Elaine Moore :
Hi, I too have Graves' disease and had radioiodine ablation. I
wouldn't recommend it. RAI stimulates the immune system worsening the
autoimmune process. Consequently, while the thyroid gland is destroyed and
hypothyroidism develops, patients are at risk for thyroid eye disease,
pretibial myxedema, and also the development of other autoimmune diseases.
RAI can also damage the salivary glands and any organs that concentrate
iodine. PTU is a safe treatment when used at the appropriate doses and it
helps the immune system heal while lowering thyroid hormone levels. It
addresses the autoimmune nature of the condition and allows patients to
move into remission. Graves' disease is a self-limiting disorder that runs
its own course. Best, Elaine
Jun 28, 2009 6:47 PM
Elaine Moore :
Hi, When you go to Charleston, you might want to consult with a
rheumatologist. With the ANA, it's not unusual for the results to be
negative when you're in remission and positive during flares. In most
autoimmune diseases, the early stages are marked by periods of remission
that alternate with periods when there are symptoms. You could ask for the
lip biopsy to help confirm a rule out a diagnosis of Sjogren's. Best,
Elaine
Jul 31, 2009 3:28 PM
Elaine Moore :
Hi, Thyroid eye disease can also cause dry eyes and flushing. You
might ask your GP to check your thyroid levels and see a rheumatologist to
further assess you for Sjogren's syndrome. Best, Elaine
Aug 27, 2009 11:06 AM
Elaine Moore :
Dear Guest, thanks for sharing your story. You might want to read my
article on multiple autoimmune syndrome,
autoimmunedisease.suite101.com/.../multiple_autoimmune_syndrome Best,
Elaine
Sep 17, 2009 2:36 PM
Guest :
I was told that I had Sjogren`s 5 years ago. I am uninsured so have not
went to a doctor for it for over a year now. I am looking for some advice
on how to stop the foot pain. My feet hurt so bad sometimes I hate to
walk. The burning of my joints and muscles I am learning to live with.
and the out breaks of rash that feels like a million bugs biting me.Ice
helps the itch. any support groups in Columbus, Ohio? anyone
with suggestions can email me at dfowlermills@yahoo.com Thank You Debbie
