Sjögren’s Syndrome and Scleroderma

Sjögren’s syndrome is a condition named after Henrik Sjögren, MD following the 1933 publication of his observations and characterization of the condition. It is the second most common autoimmune disorder, affecting 3 percent of the population 50 years or older.

In Sjögren’s syndrome, the immune system mistakenly attacks the glands that produce fluids in the body. The exact cause of the disease is not known, but it often develops together with another autoimmune disease, such as rheumatoid arthritis, lupus, or scleroderma.

Sjögren’s syndrome usually is diagnosed between ages 40 and 60, and it is nine times more common in women than men. Approximately 20 percent of scleroderma patients develop Sjögren’s syndrome.

Symptoms of Sjögren’s syndrome

Sjögren’s syndrome is a systemic disease, meaning that it affects all parts of the body.

The organs primarily affected by the syndrome are the lacrimal glands  (tear glands), and the salivary glands. Consequently, dry mouth and eyes are the most common symptoms of the disease.

The dryness in the eyes may cause grittiness, stinging, burning, redness, and sensitivity to bright lights. Lack of saliva can cause extreme dryness in the mouth and may result in the patient not being able to taste foods, as well as experiencing difficulties in swallowing and speaking.

Other symptoms include swelling, stiffness, or pain in the joints and muscles, swollen salivary glands, skin rashes, dry skin and nose, persistent dry cough, fatigue, and in women, vaginal dryness.

The lungs, kidneys, blood vessels, heart, and brain are among the organs less commonly affected by more severe types of Sjögren’s syndrome.

Treatment of Sjögren’s syndrome

There are no approved treatments that address the underlying cause of Sjögren’s syndrome, but there are treatment options to alleviate symptoms of the disease. These include eye drops to reduce inflammation, artificial tears, mouth gels and sprays to substitute saliva, nonsteroidal anti-inflammatory medicines to help with joint and muscle pain, and antifungals for infections in the mouth.

Researchers at Novartis are working on a treatment targeting the roots of Sjögren’s syndrome. An international Phase 2 clinical study (NCT02291029) is recruiting patients to assess the safety and benefits of an antibody called CZF533, which targets immune cells called B-cells that are thought to be involved in the development of the disease.

Sjögren’s syndrome and lifestyle

Certain lifestyle changes also can help people with Sjögren’s syndrome. Some of these changes include using a humidifier at home, chewing sugar-free gum to stimulate saliva production, using moisturizers for skin dryness, not smoking, avoiding smoky and windy places, and wearing protective eyewear to keep out irritants.

Adjusting dietary choices by choosing soft and smooth foods, moistening foods by adding sauces and dressings, preferring calorie-rich fluids like milkshakes and breakfast drinks, avoiding salty, acidic, and spicy foods, and sipping water regularly can help with swallowing problems.

Maintaining good dental hygiene also is important. Saliva has antibacterial properties; due to low saliva production, Sjögren’s syndrome patients are at a higher risk of infection in the mouth and developing dental problems.

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