Sjögren’s Syndrome

What is Sjögren’s syndrome?

In the early 20th century, Swedish physician Henrik Sjögren (SHOW‐gren) first described a group of women whose chronic arthritis was accompanied by dry eyes and dry mouth. Today rheumatologists know more about the syndrome that is named for Sjögren and most significantly for patients can provide advice about how to live with it.

Sjögren’s syndrome is an inflammatory disease that can affect many different parts of the body, but most often affects the tear and saliva glands. Patients with this condition may notice irritation, a gritty feeling, or painful burning in the eyes. Dry mouth or difficulty eating dry foods, and swelling of the glands around the face and neck are also common. Some patients experience dryness of other mucous membranes (such as the nasal passages, throat, and vagina) and skin.

“Primary” Sjögren’s syndrome occurs in people with no other rheumatologic disease. “Secondary” Sjögren’s occurs in people who do have another rheumatologic disease, most often systemic lupus erythematosus and rheumatoid arthritis.

Most of the complications of Sjögren’s syndrome occur because of decreased tears and saliva. Patients with dry eyes are at increased risk for infections around the eye and may have damage to the cornea. Dry mouth may cause an increase in dental decay, gingivitis (gum inflammation), and oral yeast infections (thrush) that may cause pain and burning. Some patients have episodes of painful swelling in the saliva glands around the face.

Complications in other parts of the body occur rarely in patients with Sjögren’s syndrome. Pain and stiffness in the joints with mild swelling may occur in some patients, even in those without rheumatoid arthritis or lupus. Rashes on the arms and legs related to inflammation in small blood vessels and inflammation in the lungs, liver, and kidney may occur rarely and be difficult to diagnose. Neurological complications that cause symptoms such as numbness, tingling, and weakness have also been described in some patients.

What causes Sjögren’s syndrome?

The cause of Sjögren’s syndrome is not known, but it is considered an autoimmune disorder. People with this disease have abnormal proteins in their blood suggesting that their immune system, which normally functions to protect the body against cancers and invading infections, is reacting against their own tissue. The decreased production of tears and saliva seen in Sjögren’s syndrome occurs when the glands that produce these fluids are damaged by inflammation. Research suggests that genetic factors and possibly viral infections may predispose people to developing this condition.

Who gets Sjögren’s syndrome?

Between 400,00 and 3.1 million adults have Sjögren’s syndrome. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It affects 10 times as many women as men. About half of affected patients also have rheumatoid arthritis or other connective tissue diseases, such as lupus.