Symptoms
The two leading symptoms of Sjögren’s syndrome (pronounced “Schögren”) are dry mouth and dry eyes with associated symptoms such as conjunctivitis, difficulty swallowing and speaking, gingivitis, and tooth decay. The nose, throat, skin, lips and vagina are also frequently dry. In addition, many other organs may be affected less frequently and include muscle and joint pain, skin rashes, digestive problems and fatigue. These are referred to as extraglandular sympoms. Sjögren’s syndrome may be accompanied by other autoimmune diseases, and patients are susceptible to lymphoma (cancer of the lymph glands). The disease is named after Henrik Sjögren, a Swedish ophthalmologist who described it in the 1930s.
Causes
Sjögren’s syndrome is a chronic autoimmune disease in which the immune system targets the patient’s own exocrine glands, particularly the salivary and lacrimal glands. Typical is a lymphocytic infiltration. Mainly middle-aged women are affected. The exact trigger is not precisely known, and the development is multifactorial (genetics, hormones, immune and environmental factors).
- Primary Sjögren’s syndrome: occurrence in healthy individuals.
- Secondary Sjögren’s syndrome: association with other autoimmune diseases such as rheumatoid arthritis or lupus erythematosus.
Diagnosis
The diagnosis is made in medical treatment. The specialists for the disease are rheumatologists. Because the clinical picture is nonspecific and corresponding complaints can also have other causes, months to years often pass before patients receive the correct diagnosis.
Nonpharmacologic treatment
- Drink plenty of fluids
- Humidify the air
- Good oral hygiene and dental care to prevent diseases of the teeth and gums.
- Avoid smoking and alcohol
- Physical exercise for muscle and joint pain
Drug treatment
Currently, no causal treatment exists. However, the symptoms can be alleviated with various medications. Dry mouth remedies:
- Special mouth gels, mouth sprays and mouth rinses moisten the dry mouth and act as saliva substitutes. Supportive candies, lozenges and chewing gum with the anticariogenic xylitol can be used.
- Anetholtrithione (Sulfarlem S25) is an older remedy for dry mouth and dry eyes, administered in the form of dragées.
Parasympathomimetics:
- Pilocarpine tablets (Salagen) and cevimelin (not commercially available in many countries) have cholinergic properties and promote secretion of exocrine glands.
Tear substitutes:
- The eye drops or eye gels moisturize dry eyes and serve as a substitute for the missing tear fluid. Products without preservatives should be preferred. For the treatment of dry eyes, other drugs such as eye ointments or ciclosporin eye drops are available see under dry eyes.
Analgesics:
- Such as non-steroidal anti-inflammatory drugs and paracetamol are used to treat muscle and joint pain.
Immunomodulators:
- Such as hydroxychloroquine, methotrexate, cyclophosphamide, azathioprine, ciclosporin, glucocorticoids, and biologics (e.g., rituximab) are used primarily off-label for extraglandular manifestations.
