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Sjögren's syndrome
1.
2. 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).
4. The cause of Sjögren's syndrome is not exactly
known.
Maybe by a combination of genetic and
environmental factors.
Only the presence of the gene doesn’t cause
Sjögren's 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.
5. Although Sjögren's occurs in all age groups in
men and women, it is most common in women.
Nine out of ten Sjögren's patient are women
and the average age of onset is after the
menopause.
6. It occurs in two basic forms:
Primary Sjögren's syndrome:-
◦ The disease by itself, not associated with any
other illness.
Secondary Sjögren's syndrome:-
◦ The presence of another autoimmune disease
such as rheumatoid arthritis, vasculitis, etc.
7. The hall mark symptom
of this disease is
generalized dryness.
Dryness of :
Eyes
(Keratoconjunctivitis
Mouth (xerostomia)
sicca)
8. The major signs and
symptoms are:
Dryness of skin.
Dryness of eyes.
Dryness of nose.
Dryness of vagina.
Rheumatoid arthritis.
9. Other minor signs and
symptoms incude:
Gastrointestinal problems.
Inflammation of lungs,
kidneys, liver or pancreas.
Ache or pain in the muscles
and joints.
10. The secondary Sjögren's syndrome can be
detected along with the other common
autoimmune disease such as rheumatoid
arthritis
The diagnosis for primary Sjögren's
syndrome include:
Blood test.
Slit lamp examination.
Ultrasound examination of the salivary
glands.
Schirmer’s test.
11. To determine the levels of antibodies.
Presence of antibodies such as anti-nuclear
antibody (ANA) and rheumatoid factor is
positive result for the test.
13. Ultrasound examination of the
salivary glands is the simplest
confirmatory test.
The normal parotid gland
looks homogenous under
ultrasound examination.
That of a Sjögren's patient is non-homogenous.
14. 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 Sjögren's syndrome.
15. 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.
16. There is no surgery available for treating dry
skin.
Some moisture providing
substitutes are:
Heavy moisturizers.
Use of sunscreen with at
least SPF 15.
17. Artificial tears.
◦ To provide moisture to the eyes.
◦ Cyclosporine eye drops reduce
inflammation of tear glands.
Moisture chamber spectacles.
18. Plugging of tear ducts.
◦ It reduces the mount of tears
drained from the eye.
Collagen or silicone plugs are
inserted into the ducts for a
temporary closure.
Collagen plug eventually dissolves but
silicone plug stays until they fall out or are
removed.
19. Pilocarpine (drug stimulating saliva).
Lubricants for vaginal dryness such as KY
Jelly, estrogen creams etc.
20. The Sjögren's Syndrome Foundation
is an American non-profit organization
focused on increasing research,
education and awareness for Sjögren's.
Founded in the year 1983.
Mission:
◦ Help patients cope with their Sjögren's.
◦ Increase awareness.
◦ Support Sjögren's research efforts.
It serves as the lead organization for all other
Sjögren's groups, worldwide.