Venus Williams and Sjogren's Syndrome

What is Sjogren's Syndrome, and how is it pronounced? 

Sjogren's syndrome, pronounced "Showgren's syndrome”, is a chronic systemic autoimmune disease where the body's immune system mistakenly attacks itself, destroying healthy tissues. It became widely known after Venus Williams talked about it openly in an interview. 

Dry eyes and dry mouth are the two most common symptoms that accompany Sjogren's disorder. The mucous-secreting membranes and moisture-releasing glands of the eyes and mouth are affected first, resulting in less saliva and tears. 

This condition often accompanies other immunity disorders such as rheumatoid arthritis and lupus. Usually, people diagnosed with this condition are aged over 40 years. However, one can develop Sjogren's at any age. 

Now that we understand what Sjogren's is, let us look at the symptoms of this disorder. 

Symptoms of Sjogren's Syndrome

Two main symptoms characterise Sjogren's syndrome:

Dry eyes: The eyes itch, burn, or feel gritty, like sand in them. 

Dry mouth (xerostomia) - The feeling is similar to having a 'cottonmouth' where the mouth feels dry, like it is full of cotton. 

In addition, patients with Sjogren's syndrome also suffer from one or more of the following problems: 

• Skin rashes or dry skin.
• Joint pain, swelling and stiffness.
• Swollen salivary glands - particularly the set located behind the jaw and in front of the ears.
• Vaginal dryness.
• Persistent dry cough and 
• Prolonged fatigue. 

More than 80% of people with Sjogren's syndrome suffer from fatigue and muscular and joint pains, which leads to a reduced quality of life. In 30-40% of the people with primary Sjogren's syndrome, other parts of the body, such as the brain, nerves, kidneys, heart and lungs, are also affected. 

And it doesn't stop there. Patients with Sjogren's are at 14 times more risk of developing non-Hodgkin lymphoma than the general population.

Why is Sjogren's Syndrome tricky to diagnose?

The official website of Sjogren's Foundation features a quote by Venus Williams where she says, "Sjögren's is a complex and often invisible disease. As a patient, I was misdiagnosed many times and my symptoms got progressively worse to the point where I couldn't play professional tennis anymore. I understand the daily struggles and the strength it takes to open up about your journey, but I also know the support and education that is created by awareness. I applaud all the patients who shared their stories during Sjögren's Awareness Month and everyone who is living with this disease. Your stories have inspired me, and I encourage all patients to continue to talk about their disease and make their health a priority. You are not alone."

The critical point you, as doctors, must notice in the message is the following phrase:

"Sjögren's is a complex and often invisible disease. As a patient, I was misdiagnosed many times…."

So what makes Sjogren's Syndrome such a complex disease to diagnose? Let's find out. 

The general symptoms of this disease, such as unexplained fever, involuntary weight loss, fatigue, pain, and dryness, are not specific to this syndrome alone and can potentially delay the diagnosis for many years. 

The similarity of symptoms presents a challenge for doctors and makes Sjogren's difficult to diagnose and manage. 

Before we move to the next section, here is a video on “Sjogren’s Syndrome - Investigations” by Dr Nikhil Gupta that you must watch. This lecture will clear all your doubts regarding this challenging disorder.

Next, other symptoms, such as the inflammation of the muscles, joints, skin, nervous system, kidney or lungs, can be misleading because they mimic other diseases such as rheumatoid arthritis, lupus or multiple sclerosis.  

Further, other non-autoimmune conditions, such as perimenopause, endocrine disorders, or fibromyalgia, present similar conditions and must be differentiated from primary Sjogren's syndrome. 

That leads us to the following question: 

How can one suspect Sjogren's Syndrome during a diagnosis? 

As a doctor, primary Sjogren's syndrome should be suspected in a patient when they display dryness of the mouth and eyes, joint pain, and fatigue, with or without symptoms of systemic organ complication. 

After that, let us look at the causes behind Sjogren's syndrome. 

What causes Sjogren's Syndrome?

Experts have yet to determine the exact cause of Sjogren's syndrome. One suspected cause is faulty genes that put patients at risk. The disease needs a triggering mechanism, such as an infection, to start. 

For example, if you have a faulty gene with a propensity for developing Sjogren's syndrome and you catch an infection, your immune system will swing into action immediately. 

However, instead of attacking the bacteria or virus, your white blood cells will attack healthy cells in the glands that make saliva and tears. 

People over 40 and women, in particular, are more likely to develop Sjogren's. 

Finally, let us look at what medical science says about treatment. 

Treatment for Sjogren's Syndrome

Currently, there is no cure for this syndrome. The best you can do as a doctor is to manage the symptoms. Moreover, the small list of treatment options makes managing this disease challenging. 

For example, fatigue is difficult to treat. Conditions such as hypothyroidism and obstructive sleep apnoea also cause fatigue and must be ruled out. 

To tackle dryness, doctors can prescribe artificial tears or eye drops to lubricate dry eyes. Regular checkups and fluoride treatments are recommended for dry mouth, in addition to using sugar-free chewing gum or lozenges to stimulate saliva production. 

Also read:

And that’s everything about Sjogren’s Syndrome, the ‘invisible’ disease that Venus Williams suffered through for a long time. 

Many people are suffering through Sjogren’s without being aware of it because they have not been diagnosed properly. We bring such interesting and relatively unknown diseases to you so that you are pre-informed and equipped to make the correct diagnosis. 

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