Sjögren's Syndrome: Lessons from Venus Williams
Dry eyes, dry mouth, and debilitating fatigue, how tennis champion Venus Williams brought attention to this often-missed autoimmune disease.
Like many, I was inspired watching the Williams sisters rise to the top of tennis. So it came as a surprise when Venus Williams withdrew from the 2011 US Open after being diagnosed with an autoimmune disease. In interviews she described years of extreme fatigue, dry eyes, dry mouth, and joint pain and swelling in her hands, trouble lifting her racket, heaviness and numbness in her arms, and shortness of breath. She had struggled with these symptoms long before finally receiving a diagnosis of Sjögren's syndrome.
Sjögren's is a disorder of the immune system that affects the glands' ability to produce saliva and tears. Primary Sjögren's occurs without another rheumatic disease (autoimmune thyroid disease, or Hashimoto's, can be seen alongside it), while secondary Sjögren's can occur with lupus and rheumatoid arthritis. It can affect anyone at any age but most commonly affects women in their forties and older.
The hallmark 'sicca' symptoms are dry eyes and dry mouth (xerophthalmia and xerostomia), caused by decreased tear and saliva production. Patients describe a 'gritty' sensation in the eyes and a 'cotton ball' feeling in the mouth, and often feel thirsty despite constant hydration. Debilitating fatigue, weakness, and generalized pain are common. Other symptoms include low-grade fevers, parotid gland swelling, joint pain and stiffness, muscle aches, skin dryness or vasculitis rashes, nasal dryness, chronic dry cough, Raynaud's phenomenon, vaginal dryness, reflux, and difficulty swallowing.
Because it mimics other diseases, Sjögren's can be hard to diagnose. A rheumatologist checks inflammation markers, blood counts, and specific antibodies (ANA, anti-SSA/SSB, rheumatoid factor, immunoglobulins). An ophthalmologist may perform a Schirmer tear test, and salivary gland function tests or a minor salivary gland (lip) biopsy may be used. Complications can include corneal damage, eye infections, dental cavities, oral thrush, and inflammation of the lungs, heart, liver, kidneys, thyroid, joints, skin, and nerves. Importantly, a small percentage of patients can develop lymphoma, so regular follow-up and blood tests are essential.
There is no cure, but treatment targets symptom relief: artificial tears and lubricants (or cyclosporine eye drops like Restasis for more severe dryness), saliva-stimulating medications (pilocarpine, cevimeline), NSAIDs for joint pain, antifungals for thrush, and immunosuppressants like hydroxychloroquine and methotrexate for systemic symptoms. Lifestyle helps too: stop smoking, increase water, limit alcohol and caffeine, humidify your environment, brush frequently, and get enough sleep. Venus adopted a plant-based diet and went on to win the 2012 Wimbledon doubles title and compete for years, an inspiring reminder that people with Sjögren's can lead healthy, productive lives.
Dr. Maggie Cadet, MD