Sjögren’s syndrome

Clinical picture

Sjögren’s syndrome is a long-term autoimmune disorder that affects parts of the body that produce fluids such as tears and spit (saliva). An autoimmune disease (auto = self, immune = defence) is a condition in which your immune system attacks cells and substances that are native to your own body. White blood cells (lymphocytes) enter the glands that produce fluids, such as tear-glands and salivary glands. The resulting inflammation causes damage to these glands and produce less gland fluid, leading to dry eyes and dry mouth. Sjögren’s syndrome is also known as sicca syndrome (sicca = dry).

Sjögren’s syndrome is named after Henrik Sjögren, a Swedish ophthalmologist, who described this disorder in 1933. Sjögren’s syndrome is classified as a systemic disease in the subgroup of inflammatory rheumatism, together with systemic lupus erythematoses and scleroderma. The incidence in women is ten times higher as in men, with an onset usually between 30 and 60 years of age. People with the primary type of Sjögren’s syndrome have a greater risk of developing a type of cancer called non-Hodgkin lymphoma.

Sjögren’s syndrome can be divided in a primary and a secondary form:

• Primary Sjögren’s syndrome when it occurs by itself.
• Secondary Sjögren’s syndrome when associated with another connective tissue disease, such as systemic lupus erythematoses or rheumatoid arthritis.

Symptoms

Sjögren’s syndrome is associated with a lot of symptoms. The most common symptoms are:

• Dry eyes: itchy, irritated and sore eyes. Eyes are easily irritated by dry air, air conditioning, cigarette smoke and bright light.
• Dry mouth: dry lips, diminished taste and smell, difficulty chewing, swallowing and speaking. The salivary glands may be enlarged due to infection. Tooth decay and fungal infections are also more common than in the general population.
• Fatigue: shortness of breath.

Other signs and symptoms of Sjögren’s syndrome:

• dry, irritated throat, and cough
• heartburn (acidity of the stomach)
• dry skin
• vaginal dryness
• muscle pain
• joint pain
• Raynaud’s phenomenon (paleness and numbness of the fingers or toes triggered by cold or emotional stress)
• scleroderma
• bladder infection
• sensory disturbances, particularly the sole of the foot

Cause

Sjögren’s syndrome is an autoimmune disorder caused by an abnormal reaction of the immune system to its own body. There is evidence for a role of viral infections, hormones and genetic factors in the pathogenesis of this autoimmune disease. Why some people are more susceptible than others, is yet unknown.

Sjögren’s syndrome can exist as a clinically isolated syndrome, or as a consequence of another disorder such as rheumatoid arthritis. Rheumatoid arthritis is, like Sjögren’s syndrome, an autoimmune disorder, in which the immune system attacks the own joints. Patients often have more than autoimmune disease.

Diagnosis

The diagnosis of Sjogren’s syndrome is made based on the pattern of signs and symptoms, physical examination and blood tests. The following tests may be used to diagnose Sjogren’s syndrome:

• Structured patient history, taking into account the medical history of the patient.
• Physical examination.
• Schirmer’s test: to determine whether the eyes produce enough tears to keep them moist.
• Biopsy (remove and examine a small piece of the salivary gland).
• Blood tests to exclude other disorders, estimate the severity of the disease and the risk of possible complications.

Treatment

Sjogren’s syndrome is a lifelong condition. Sjögren’s syndrome cannot be cured, but proper treatment can help to relieve symptoms:

• Eye drops can prevent dry eyes.
• To ease dry mouth, saliva substitutes (mucin-containing oral spray) can be used. It also helps to drink water regularly and chew sugar-free gum.
• Pilocarpine tablets for patients whose saliva production can still be stimulated.
• Moisturising creams for dry skin.
• Lubricants for vaginal dryness.
• Painkillers, nonsteroidal anti-inflammatory medications (NSAIDs), or prednisone can reduce joint swelling and stiffness, and muscle aches.
• Sufficient physical exercise can reduce fatigue.

Furthermore, targeted therapy for Sjorgen’s syndrome is an active area of research that may allow for more treatment pathways in the future.

Additional information

Patient organisations

Sjögren’s Foundation

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