Diagnosis of Sjögrens Syndrome

Sjögren’s syndrome is often diagnosed late in the process which means people with Sjögrens often suffer for years before diagnosis.

The delay in diagnosis is partly due to lack of information and understanding of the condition by the general public and many  health professionals but also because of the many  and varied symptoms a lot of which have possible other causes.  The symptoms may present one by one, on and off over the years and it may not be until several are present at once that Sjögrens is considered.  It is not uncommon for people with Sjögrens to have been mistakenly told they are suffering from some sort of psychological condition or given the impression that they are neurotic, implying the symptoms are more imaginary and related to stress and anxiety.  We do know stress will exacerbate auto immune disease as it does many disease processes and cause it to flare up….  but it is not the cause.

Health professionals who most often diagnosis Sjögrens include:

  • Rheumatologists
  • Dentists
  • Opthalmologists/Optometrists
  • General Practitioners

Diagnosis is usually clear from the symptom combination:

  • Dry eyes; Dry mouth; Fatigue; Muscle and joint pains, and the association with other connective tissue disorders.

Symptom criteria for diagnosis:

Dry eyes (at least one of the following)

  • Persistent, troublesome dry eyes every day for longer than 3 months
  • Recurrent sensation of sand or gravel in the eyes
  • Use of a tear substitute more than three times a day

Dry Mouth (at least one of the following)

  • Feeling of dry mouth every day for at least 3 months
  • Recurrent feeling of swollen salivary glands as an adult
  • Need to drink liquids to aid in swallowing dry foods

Tests used with may be used to confirm the diagnosis:

BLOOD TESTS:

Antibody Tests Specific to Sjögren’s Syndrome:

Anti -SS-AA  (Anti-Ro) and Anti -SS-B (Anti-La);

About 75% of people  with primary Sjögren’s syndrome will have Anti SS-A and Anti SS-B  antibodies and as  they rarely are found in other conditions, it is a very  useful in test for diagnosing primary Sjögren’s syndrome.

Other blood tests your doctor is likely to test are;

  • Autoantibody screen including ANA
  • Rheumatoid factor (RF) and Anti -CCP(if rheumatoid arthritis is suspected) or IG
  • Inflammatory markers CRP and ESR ( these tests may reflect the severity of the disease activity)
  • Liver function tests and kidney(renal) tests i.e. creatinine and electrolytes ..both liver and kidney may be  involved in autoimmune disease
  • Thyroid tests..  as autoimmune thyroid disease is associated with secondary sjogrens

EYE TESTS

Slit lamp examination – performed by an eye specialist (ophthalmologist or optometrist) The slit lamp allows the specialist to examine the tear film and outer layers of the eye under magnification. The tear film is examined using a Rose-Bengal stain.  In Sjögrens the tear film is depleted and of abnormal quality-it breaks up quickly allowing drying of the surface of the eye which leads to damage of the tissues.

Schirmer’s test – Is when a small piece of sterile blotting paper (or filter paper) is placed in each eye, under the lower lid, after the surface of the eye has been numbed with anaesthetic drops. The eyes are then closed for 5 minutes while the the paper collects any tears secreted.  A  Normal result is 10mm or more.

Fluorescene drops (bright orange dye) is sometimes used to test tear production and drainage.  In the normal eye these should be washed away by tears down tear ducts into the nose within 2 minutes.  In dry eye conditions the stain will remain for longer.

DRY MOUTH TESTS

Saliva production measurement – The simplest way to do this is to:
Collect all saliva produced over 15 minutes (with no stimulation from chewing)  and if this is less than or equal to 1.5ml it is diagnostic of xerostermia

Another method studied is:
The  person to be tested spits all saliva produced in 5 minutes into a measuring container,
then  chews on a piece of dental (paraffin) wax for 5 minutes during which time all saliva produced is spat into another container;
then chews on tasty sugarfree gum or candy for 5 minutes and again spits all saliva into a container.

In a normal person at each step there should be a significant  increase in amount of saliva produced. In xerostermia (dry mouth) there is virtually no change in amounts between the 3 stages.

Scintigraphy of salivary glands –
During  scintigraphy a small quantity of radio-active material is injected into the bloodsteam which is taken up by the salivary glands and then secreted in the saliva into the mouth.  A special digital camera takes images at frequent intervals which shows the progression of the flow throught the ducts.

Sialography of the parotid gland is sometimes performed.

Sometimes MRI scans and ultrasound scans are used to diagnose lumps on salivary glands or lymph nodes which are suspected to be lymphoma

Lip biopsy

Occasionally a lip biopsy may be performed –this was previously thought to be the “gold standard” for diagnosis of Sjögrens however it is now only performed if  the diagnosis is still unclear after all other tests. Fortunately diagnosis is  usually able to be made on the basis of presenting symptoms of Sjögrens and blood tests specific to Sjögrens.

The test is performed under local anaesthetic and a small sample of the minor salivary glands inside the mouth just below the lip is taken for examination of the cells under the microscope (histology). In Sjögrens lymphocytic infiltration and destruction of the glands tissue is seen.

PATIENT INFORMATION: Sjögren’s syndrome – Paul Cramer, MD Mead Hospital, Bristol, England