Sjogren's syndrome

Definition

Autoimmune disease attacking exocrine glands

Epidemiology

90% are women, 4 million sufferers in the USA.

Pathophysiology

As with all automimmune diseases, nobody really knows. Anti-nuclear antibody (ANA) and rheumatoid factor are both associated with it but then they're associated with everything rheumatological.

The main thing is to think DRY, DRY, DRY, DRY, DRY, DRY!

Risk Factors

Having rheumatoid arthritis or a whole spectrum of other autoimmune diseases.

Clinical Features

An exocrine gland secretes liquid into a duct. Anywhere there is a duct through which stuff is secreted, less stuff is now secreted. These are the main places it affects:

Eyes

Sjogren was an ophthalmologist and the main thing he noticed was dry eyes. You can develop corneal ulcers

Salivary

You also get a dry mouth xerostomia which gives ulcers on the inside of the mouth and increases the likelihood of dental caries (saliva is protective).

Other organs

• Skin, nasal and vaginal dryness all occur.
• Other organs potentially affected are kidney, liver, blood vessels, pancreas, nerves and brain

Investigations

• ANA
• Rheumatoid factor
• Schimmer's test - a piece of paper is held under the eyelid. If it's not wet enough (<5mm), this is diagnostic
• USS of salivary gland

There are whole host of tests but these are the main ones. A combination of clinical features and investigations is used in the diagnosis.

Management

There is no definitive management. The disease progression can be slowed and halted but not reversed.

Eyes

• Artifical tears
• Punctal plugs (the puncta of the eyelid are the outflow tract for tears. You block them off.)

Mouth

You need to see your dentist regularly. They do some stuff. Ask a dentist if you care.