Sjögren’s Syndrome - What Is It?

Sjögren’s Syndrome — Introduction

Ever since tennis champion Venus Williams dropped out of the U.S. Open tennis competition back in 2011, interest has trended upward for the incurable immune system disease that threatened to end her career.

Despite the diagnosis of Sjögren’s syndrome, which Venus has been battling for more than six years, she continues to play today like the champion she is. Media outlets from People Magazine and Essence to The Dr. Oz Show follow her progress and celebrate her success. Not long ago she won the Wimbledon women’s doubles championship with her sister Serena in July of 2016. Later that year she captured an Olympic mixed-doubles silver medal in Rio de Janero with partner Rajeev Ram. In January 2017 she battled all the way to the finals of the Australian Open. At the age of 37 in July 2017 she reached the Wimbledon singles final. Most recently in September 2017 Venus Williams became the oldest woman ever to reach the U. S. Open women’s semifinals. She shows few signs of slowing down now despite her Sjögren’s syndrome diagnosis.

With a condition as complex as Sjögren’s, there is an abundance of information circulating — some of it conflicting or not very well documented. Many questions still surround the diagnosis:

What is the real truth about Sjögren’s syndrome?
Do we know the cause(s)?
Are effective treatments available now?
What is the likely prognosis for an individual diagnosed with Sjögren’s today?
Which current research efforts are likely to improve the outcome?

Read on to learn more about challenges and opportunities confronting Venus Williams and the other 500,000 to 4 million Sjögren’s patients in the United States today.

Sjögren’s Syndrome — Definition and Epidemiology

Sjögren’s (SHOW-grins) syndrome is a chronic (persistent, long-lasting), systemic (affects the entire body), autoimmune disease that causes dryness of the eyes, mouth and other body parts. Along with those symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies and lymphomas.

By definition an autoimmune disease results from the abnormal production of extra antibodies in the blood. The immune system then mistakenly directs these antibodies to attack healthy tissue, which in turn leads to inflammation. Specifically with Sjögren’s syndrome the lymphocytes (infection-fighting cells of the immune system) attack normal cells of exocrine glands (glands whose secretions pass through a duct directly to the outside of the body) that produce moisture in the eyes, mouth and other tissues. This action can damage the glands to a point where eventually they are unable to produce adequate moisture.

Today the Sjögren’s Syndrome Foundation estimates that as many as four million Americans are living with this disease. It is the second most common autoimmune disease (after rheumatoid arthritis and ahead of lupus). Studies of the epidemiology of Sjögren’s syndrome report a prevalence of between 0.05 and 4.8 percent of the population. Prevalence rises with age. Ninety percent of people with the condition are female. Most individuals are 40 years or older when diagnosed; although, Sjögren’s can occur in all age groups, including children, and in both sexes and all races.

Although scientists do not know the exact cause of Sjögren’s syndrome, they think it may start with exposure of susceptible genes to a viral or bacterial antigen. There also seems to be familial predisposition associated with the condition. Chances of a person developing Sjögren’s syndrome are greater if close blood relatives have a history of it or of another autoimmune diseases.

Sjögren’s Syndrome — Classification

The disease is usually classified according to the following distinctions, which occur in almost equal numbers:

Primary Sjögren’s — occurs by itself (not associated with any pre-existing condition)
Secondary Sjögren’s — occurs after another connective tissue disease (e.g., rheumatoid arthritis, lupus, scleroderma or myositis) is already present

note: this classification system does not necessarily correlate with the severity of symptoms or complications. Primary Sjögren’s and Secondary Sjögren’s patients all are susceptible to the same level of discomfort, complications and seriousness of the disease.

Sjögren’s Syndrome — Prognosis

Most patients with Sjögren’s syndrome remain relatively healthy, but some rare complications, including an increased risk for cancer of the lymph glands, may develop. B-cell lymphoma is 24 times more common in Sjögren’s patients than in the general population. Regular medical care and follow-up is important for all Sjögren’s sufferers.

Sjögren’s syndrome is not curable, but in many cases proper treatment helps to alleviate or minimize symptoms. Often people with Sjögren’s are able to live relatively normal lives with only minor adjustments. Treatment needs to be individualized for each person and determined by which parts of the body are affected. Typically the program will focus on eliminating or managing symptoms as well as preventing and treating long-term complications.

note: treatments often do not completely eradicate the symptoms of dryness.

For more information about Sjögren’s syndrome, please read Vitamin Insider posts about “Symptoms/Cause(s)/Diagnostic Techniques” and “Current Treatments and Research for the Future.”