Sjögren's syndrome

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Sjögren's syndrome

  1. 1.  Pronounced as “shohgrinz syndrome”. It is an autoimmune disease in which immune cells attack and destroy the exocrine glands. It is the second most common autoimmune disease after SLE (Systemic lupus erythematous).
  2. 2. Tennis champion VENUS WILLIAMSwas diagnosed with primarySjögrens syndrome.
  3. 3.  The cause of Sjögrens syndrome is not exactly known. Maybe by a combination of genetic and environmental factors. Only the presence of the gene doesn’t cause Sjögrens syndrome. An external trigger is said to activate the immune system. The immune system responds even when there are no foreign substances to fight off. This inflammatory response causes the body’s white blood cells to attack and destroy certain moisture producing glands.
  4. 4.  Although Sjögrens occurs in all age groups in men and women, it is most common in women. Nine out of ten Sjögrens patient are women and the average age of onset is after the menopause.
  5. 5.  It occurs in two basic forms: Primary Sjögrens syndrome:- ◦ The disease by itself, not associated with any other illness. Secondary Sjögrens syndrome:- ◦ The presence of another autoimmune disease such as rheumatoid arthritis, vasculitis, etc.
  6. 6.  The hall mark symptom of this disease is generalized dryness. Dryness of : Eyes (Keratoconjunctivitis Mouth (xerostomia) sicca)
  7. 7. The major signs and symptoms are: Dryness of skin. Dryness of eyes. Dryness of nose. Dryness of vagina. Rheumatoid arthritis.
  8. 8. Other minor signs and symptoms incude: Gastrointestinal problems. Inflammation of lungs, kidneys, liver or pancreas. Ache or pain in the muscles and joints.
  9. 9. The secondary Sjögrens syndrome can be detected along with the other common autoimmune disease such as rheumatoid arthritis The diagnosis for primary Sjögrens syndrome include: Blood test. Slit lamp examination. Ultrasound examination of the salivary glands. Schirmer’s test.
  10. 10.  To determine the levels of antibodies. Presence of antibodies such as anti-nuclear antibody (ANA) and rheumatoid factor is positive result for the test.
  11. 11. Ophthalmologist usesequipment toexamine the eye.It helps in knowing howsevere is the drynessand inflammation.
  12. 12.  Ultrasound examination of thesalivary glands is the simplestconfirmatory test. The normal parotid glandlooks homogenous underultrasound examination. That of a Sjögrens patient is non-homogenous.
  13. 13.  Schirmer’s test measures the production of tears. Procedure: ◦ Strip of filter paper is held inside the lower eyelid for 5mins. ◦ Wetness is measured using ruler. ◦ Production of less than 5 mL of liquid is an indicative of Sjögrens syndrome.
  14. 14.  Alternate test: ◦ The patient is asked to spit into a test tube every minute for 15 minutes. ◦ A resultant collection of less than 1.5 mL is considered a positive result.
  15. 15.  There is no surgery available for treating dryskin.Some moisture providingsubstitutes are: Heavy moisturizers. Use of sunscreen with atleast SPF 15.
  16. 16.  Artificial tears. ◦ To provide moisture to the eyes. ◦ Cyclosporine eye drops reduce inflammation of tear glands. Moisture chamber spectacles.
  17. 17.  Plugging of tear ducts. ◦ It reduces the mount of tears drained from the eye. Collagen or silicone plugs areinserted into the ducts for atemporary closure. Collagen plug eventually dissolves but silicone plug stays until they fall out or are removed.
  18. 18.  Pilocarpine (drug stimulating saliva). Lubricants for vaginal dryness such as KY Jelly, estrogen creams etc.
  19. 19.  The Sjögrens Syndrome Foundationis an American non-profit organization focused on increasing research,education and awareness for Sjögrens. Founded in the year 1983. Mission: ◦ Help patients cope with their Sjögrens. ◦ Increase awareness. ◦ Support Sjögrens research efforts. It serves as the lead organization for all other Sjögrens groups, worldwide.

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