Sjogren's Syndrome

Sjogren's syndrome is an autoimmune disorder that decreases the amount of moisture produced by the eyes and saliva by salivary glands. It is named after Swedish eye doctor Henrik Sjogren, who first described the condition.

Other names #

Also known as Sicca syndrome, Mikulicz’s disease, Dacryosialoadenopathia atrophicans, Gougerot-Houwer-Sjogren syndrome, Gougerot-Sjogren syndrome, Keratoconjunctivitis sicca, Keratoconjunctivitis sicca-xerostomia, Secreto-inhibitor-xerodermostenosis and Sjogren-Gougerot syndrome

Causes #

• Sjogren’s syndrome is mostly thought to be an autoimmune disease, in which the immune system attacks the healthy cells instead of the diseased ones as a result of some triggers.

• In this syndrome, the immune system attacks the ophthalmic glands in the eyes and salivary glands in the mouth.

• Though the exact cause of the syndrome is still unclear, Sjogren’s syndrome is thought to be a result of a combination of genetic and environmental factors.

• According to several researchers, many genes affect the risk of developing Sjogren’s syndrome, but that development of the condition may be triggered by something in the environment as well.

• In particular, viral or bacterial infections can activate the immune system, and cause the development of Sjogren’s syndrome in susceptible individuals.

Types #

Sjogren’s syndrome is classified into two forms, namely:

• Primary form: In this type, the syndrome is not associated with any type of rheumatic disease. This form develops as a result of any other health condition.

• Secondary form: This form is associated with other rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma (hardening and tightening of the skin), polymyositis (muscle weakness affecting both sides of the body), or psoriatic arthritis (arthritis associated with psoriasis - red patches of skin topped with silvery scales).

Symptoms #

• Dryness of the eyes, mouth, and skin

• Dryness of the vagina

• Tiredness/fatigue

• Pain in the muscles and joints

• Swelling of the salivary glands

• Rashes on the skin, especially after sun exposure

• Dry nasal passages and throat along with a dry cough

• Acid reflux

• Swelling of the glands around the face and neck

• Trouble sleeping

• Poor concentration and memory problems

• Numbness, tingling, and weakness, especially in the extremities

• Shortness of breath or trouble breathing

• Muscle weakness

Diagnosis #

Schirmer test #

It is done to estimate the functioning of the lacrimal glands to measure the production of tears. A wetting paper strip of ≤ 5 mm is placed under the lower lid for 5 mins, after which the paper is removed and checked for its moisture content.

Cornea and conjunctiva examination #

This test is done to check for any punctuate or filamentary keratitis lesions, which is a typical feature of keratoconjunctivitis sicca. It is done using a rose bengal or lissamine green stain.

Salivary analysis #

It is done to estimate the production of saliva from an unstimulated salivary gland. The value is considered normal if there is > 1.5 ml in 15 minutes.

Biopsy of minor salivary gland #

This is done to assess the presence of lymphocytic infiltrates around salivary gland epithelium which is a hallmark of Sjogren’s syndrome.

Past medical history #

If the patient has any pre-existing autoimmune disease, along with dry eyes and dry mouth, it can be concluded that the patient has developed secondary Sjogren’s syndrome.

Treatment #

Dry eyes and eye inflammation #

• Dry eyes are the most common symptoms of this syndrome. Mild to moderate cases are managed by the following:

• Use of artificial tears during the day and a gel at night.

• Hot compresses and eyelid cleansers along with gentle eyelid massage to relieve blocked oil glands in the eyelids, in case of blepharitis (eyelid inflammation).

• Eye drops prescribed by an ophthalmologist.

• In certain severe cases of dry eyes, the doctor might suggest a minor surgical procedure called punctal occlusion.

• This procedure consists of the insertion of tiny plugs into the tear duct by the ophthalmologist to block them.

• It is done to sustain tears in the eyes longer, which will help keep them moist.

Dry mouth and its related symptoms #

There are a lot of over-the-counter options that are available to relieve the symptoms of dry mouth. They are:

• Sugarless candy

• Sugar-free gum

• Artificial saliva products such as a spray or lozenge

If this does not help, the patient may consult a dentist or a general physician to get prescribed medications like:

• Pilocarpine: Pilocarpine stimulates certain nerves and glands in the body thereby increasing the production of saliva, tears, sweat, digestive juices, and other secretions.

• Cevimeline: Cevimeline also helps to increase the secretions of the exocrine glands.

Other symptoms #

Individuals with Sjogren’s syndrome often experience joint pain due to rheumatic disorders. They may require medications such as:

• Ibuprofen (nonsteroidal anti-inflammatory drugs or NSAIDs)

• Prednisone (low-dose steroids)

• Hydroxychloroquine (disease-modifying anti-rheumatic drug )

• Immunosuppressants

• Antifungals