Sunday, 17 February 2013

Sjogren's Syndrome: Facts and Information

Sjögren’s is another autoimmune disease that affects those who have lupus, or similar illnesses like rheumatoid arthritis. If you have an autoimmune disease, then you will know what I mean when I say that one autoimmune disease will bring many more of its autoimmune friends along to the party. Sjögren’s is just one of many potential 'friends'.

What is Sjögren’s exactly?

It is pronounced like Show-grens, and is another autoimmune condition; this time the immune system attacks the fluid-secreting glands in the body e.g. the tear and saliva glands.

Just like with lupus, 90% of Sjögren’s patients are female (seems like we get a raw deal in the autoimmune world!). It rarely affects young children and the statistics suggest that it is more common in women in their 30's and 40's.1

There are two types of Sjögren’s: 

Primary Sjögren’s, which does not occur with any other illnesses. This form is more likely to be more severe than the secondary version.

Secondary Sjögren’s, which develops in association with other autoimmune disorders like rheumatoid arthritis, lupus and other autoimmune conditions.

What are the symptoms of Sjögren’s?

The symptoms of Sjögren’s are wide and varied. Just like lupus, it is a condition that affects each person differently. The main two symptoms are dry eyes and dry mouth, as Sjögren’s attacks the tear and saliva glands. Contrary to popular belief, Sjögren’s does not just affect the eyes and mouth.It is a systemic disease which can affect many other body tissues, causing symptoms such as:

  • Dysphagia (difficulty swallowing) and food getting ‘stuck’ in your mouth, throat and oesophagus due to the dryness caused by Sjogren’s.
  • ‘Cotton-mouth’ i.e. very dry mouth, especially in the morning.
  • Vaginal dryness
  • Ulcers and other dental problems
  • Itchy, burning, sore eyes
  • Recurrent eye infections
  • Recurrent oral thrush and oral infections
  • Swollen eyelids
  • Fatigue
  • Sore, swollen joints
  • Digestive problems
  • Dry skin
  • Headaches
  • Feverishness
  • Swollen lymph and salivary glands 

Arthritis Research UK also adds that “Sjögren’s syndrome can sometimes cause problems with your nervous system, liver and kidneys, inflammation of your blood vessels and chest pain.”

Many of the symptoms are similar to lupus, and just like lupus, it is under-diagnosed.
Sjögren’s may also be associated with other conditions, such as Raynaud's, CREST syndrome and APS, as well as lupus and rheumatoid arthritis.2

Diagnosing Sjogren's:

Tests for Sjögren’s may include blood tests, lip biopsies, Schirmers test and other eye examinations, x-rays, salivary flow rates and others.5

Sjögren’s Syndrome Foundation describes the type of blood tests doctors use to diagnose Sjögren’s:

ANA (Anti-Nuclear Antibody)
ANAs are a group of antibodies that react against normal components of a cell nucleus. About 70% of Sjögren’s patients have a positive ANA test result.

RF (Rheumatoid Factor)
This antibody test is indicative of a most often performed for the diagnosis of rheumatoid arthritis (RA) but is positive in many rheumatic diseases. In Sjögren’s patients, 60-70% have a positive RF. 

SS-A (or Ro) and SS-B (or La)
These are the marker antibodies for Sjögren’s. Seventy percent of Sjögren’s patients are positive for SS-A and 40% are positive for SS-B (these may also found in lupus patients).

ESR (Erythrocyte Sedimentation Rate)
This test measures inflammation. An elevated ESR indicates the presence of an inflammatory disorder, including Sjögren’s.

IGs (Immunoglobulins)
These are normal blood proteins that participate in immune reactions and are usually elevated in Sjögren’s patients.6


 Eye tests: Tear break-up time and Schirmer tests

These two tests are usually carried out by an ophthalmologist.

The tear break-up time test is a measure of how effective your tear glands are. During the test, a non-toxic dye is dropped onto the surface of your eye. The distinctive colour of the dye allows the ophthalmologist to see how well your tear film is functioning and how long it takes for your tears to evaporate. This test is also called a slit lamp examination.

In the Schirmer test, small strips of blotting paper are placed under your eyelid. After five minutes, the strips are removed and then studied to see how much of the paper was soaked with tears. 7

The Schirmer Test

A blogger describes the Schirmer’s test as thus:

“A rheumatologist diagnosed my SS using a Schirmer’s Test. He placed a small, thin paper under my lower eyelid to check the dryness of the eyes. It was very simple and quick and done in his office and scored to tell the amount of dryness in the eye.

Results of a Schirmer’s test:

1. Normal which is 15 mm
2. Mild which is 14-9 mm
3. Moderate which is 8-4 mm
4. Severe which is 4 mm

It was this result of 5mm and my symptoms that confirmed the syndrome as no single test is specific in diagnosing SS. It was followed with a blood test to show antibodies.”

The Sjögren’s Foundation describes another eye test that may be used, the "Rose Bengal and Lissamine Green" test. "Eyedrops containing dyes that an eye care specialist uses to examine the surface of the eye for dry spots."

They also add that various dental tests may also be conducted:

Salivary Flow, which measures the amount of saliva produced over a certain period of time. 

Salivary scintigraphy, a nuclear medicine test that measures salivary gland function.
Salivary gland biopsy (usually in the lower lip), which confirms inflammatory cell (lymphocytic) infiltration of the minor salivary glands.8

How is Sjögren’s treated?

Treatment for Sjögren’s "differs for each person. It depends on what parts of the body are affected. Treatment will focus on getting rid of symptoms. Treatment may includes:

  • Medicines for joint or muscle pain (such as aspirin and ibuprofen)
  • Medicines that help you make more saliva
  • Medicines that suppress inflammation (such as corticosteroids)
  • Medicines that suppress the immune system.9

Treatment for dry eyes may include:

  • Artificial tears that come in different thicknesses. You may have to try a few to find the right one.
  • Eye ointments. These are thicker than artificial tears. They protect the eyes and keep them wet for several hours. They can blur your vision, so you may want to use them while you sleep.
  • Medicines to reduce inflammation in the eye. A chemical that wets the surface of the eye and keeps the natural tears from drying out so fast. It comes in a small pellet that you put in your lower eyelid. When you add eye drops, the pellet melts. This forms a film over your own tears and traps the moisture. 
  • Surgery to shut the tear ducts that drain tears from the eye.

Treatment for dry mouth may include:

  • Chewing gum or sucking on hard candy helps your glands make more saliva (sugar-free)
  • Sipping water or a sugar-free drink often to wet your mouth.
  • Using oil or petroleum-based lip balm or lipstick to help dry, cracked lips feel better.
  • Using a saliva substitute prescribed by a doctor to make the mouth feel wet.
  • Using medicine to help your mouth make more saliva.

Other Information About Sjögren’s

  • People with dry mouth can easily get mouth infections. Tell your doctor if you have white patches or red, burning areas in your mouth.10
  • Some studies indicate that those with Sjögren’s are at an increased risk of Non-Hodgkin's lymphoma, especially those with a low C4 count.3
  • Sjogren's was first 'discovered' in 1933 by the Swedish opthalmologist, Henrik Samuel Conrad Sjögren.4
  • April is Sjögren’s Awareness Month. Check out the World According to Lupus on Facebook for more information and awareness campaigns. 

I hope that you have found this information piece helpful. If you have any questions, please leave a comment and I will get back to you as soon as I can. Thank you for reading!


8-9. See 6.

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