Sjögren's

syndrome

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).

Sjögren's syndrome

Tennis champion VENUS WILLIAMS was diagnosed with primary Sjögren's syndrome.

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.

Causes of Sjögren's syndrome

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.

Victims of Sjögren's syndrome

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.

Forms of Sjögren's syndrome

The hall mark symptom of this disease is

generalized dryness. Dryness of :

Signs and symptoms

Eyes (Keratoconjunctivitis

sicca)Mouth (xerostomia)

The major signs and symptoms are:

Dryness of skin. Dryness of eyes. Dryness of nose. Dryness of vagina. Rheumatoid arthritis.

Signs and symptoms

Other minor signs and symptoms incude:

Gastrointestinal problems. Inflammation of lungs,

kidneys, liver or pancreas. Ache or pain in the muscles

and joints.

Signs and symptoms

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.

Diagnosis

To determine the levels of antibodies. Presence of antibodies such as anti-nuclear

antibody (ANA) and rheumatoid factor is positive result for the test.

Blood test

Ophthalmologist uses equipment to examine the eye.

It helps in knowing how severe is the drynessand inflammation.

Slit lamp examination

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.

Ultrasound examination

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.

Schirmer’s test

Alternate test:◦ The patient is asked to spitinto a test tube every minutefor 15 minutes.◦ A resultant collection of lessthan 1.5 mL is considereda positive result.

Schirmer’s test

There is no surgery available for treating dry

skin.Some moisture providingsubstitutes are: Heavy moisturizers. Use of sunscreen with atleast SPF 15.

Treatment for dry skin

Artificial tears.◦To provide moisture to the eyes.◦Cyclosporine eye drops reduceinflammation of tear glands.

Moisture chamber spectacles.

Treatment for dry eyes

Plugging of tear ducts.◦It reduces the mount of tears

drained from the eye. Collagen or silicone plugs are inserted into the ducts for atemporary closure. Collagen plug eventually dissolves but

silicone plug stays until they fall out or are removed.

Surgery for eyes

Pilocarpine (drug stimulating saliva). Lubricants for vaginal dryness such as KY

Jelly, estrogen creams etc.

Treatment

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.

Sjögren's Syndrome Foundation