What is Sjögren’s syndrome?
Sjögren’s syndrome is a systemic autoimmune disease in which exocrine (external) glands (such as saliva, tears, vaginal secretions, lower and upper respiratory tract glands) are primarily involved. Persistent dry mouth and eyes due to functional and structural deterioration in the salivary and lacrimal glands is the most important sign of the disease. In addition, the disease can affect many organs and systems such as the musculoskeletal system, lungs, vessels, kidneys, bladder, lymph node, liver, pancreas, gastrointestinal and nervous system. When the disease is found alone, ‘primary Sjögren’s syndrome’; When it is associated with other autoimmune rheumatic diseases such as rheumatoid arthritis and lupus, it is called ‘secondary Sjögren’s syndrome’.
Who gets Sjogren’s syndrome?
-Although it can be seen at any age, it is more common in people older than 40 years of age. It is usually seen in one out of every hundred people. Its incidence approaches 5% with age.
-It is 9-10 times more common in women than men.
-It is associated with other autoimmune diseases such as rheumatoid arthritis, lupus, Hashimoto’s thyroiditis, primary biliary cirrhosis.
What are the causes of Sjögren’s syndrome?
Sjögren’s syndrome is an autoimmune disease. The immune system (immune system) is our body’s defense system. By producing certain proteins (such as immunoglobulin) or through its cells, it protects us from foreign microbes and cancer. In Sjögren’s, the immune system goes astray, and when the body fights against its own cellular structures, inflammation and related damage occur in many organs and systems. It is unknown what causes this. However, as in all autoimmune systemic connective tissue diseases, in Sjögren’s, abnormal immune response occurs as a result of the interaction of the individual’s appropriate genetic makeup and environmental factors (infections, drugs, toxins, etc.).
What are the signs of Sjögren’s syndrome?
-Dry mouth; There is dry mouth that has been going on for at least three months. Patients complain of difficulty in swallowing solid-starchy foods and without sticking their tongue to the palate while speaking. Saliva is very important for oral and dental health; prevents harmful bacteria from settling. Due to dry mouth; It is accompanied by diseases such as frequent tooth and gum problems, fungal plaques in the mouth.
-Dry eyes; There is dry eye for at least three months. There is a feeling of stinging, burning, and sand in the eyes. He has a history of using artificial tear drops and gel. Tears almost form a film layer in the eye and protect the eye from foreign bodies and infections with some of the substances it contains. With severe dry eye, diseases such as keratitis or corneal ulcers develop.
-Painless swelling of the salivary glands in front of the ear and under the chin (parotid or submandibular), usually bilateral (like mumps, but can be unilateral or bilateral in Sjögren’s and is painless unlike mumps).
-Pain, swelling or morning stiffness in the joints (especially in small joints such as the hand joints),
-Dryness of the skin or skin rash,
-Vaginal dryness,
-Continuous dry cough,
-Long-term fatigue,
-Frequent upper or lower respiratory tract infections (with decreased respiratory tract secretions-such as sinusitis, pharyngitis, laryngitis, pneumonia, bronchiectasis)
What are the complications of Sjögren’s syndrome?
-Tooth decays, gum diseases, bad breath, fungal infection in the mouth; Sometimes it can extend to the esophagus.
-Due to dry eye, sensitivity to light, blurred vision, ulcers on the cornea.
-Infection due to vaginal dryness, discharge, painful sexual intercourse, etc.
-Pharyngitis, laryngitis, frequent upper and lower respiratory tract infections
-Less; It causes problems with the lungs (such as diffuse interstitial lung disease, pleural effusion), kidneys (such as renal tubular acidosis, glomeronephritis), liver (such as hepatitis, cirrhosis).
-Cystitis,
-Only a small percentage of pregnant women with Sjögren’s syndrome pass on to the baby with autoantibodies (anti-SSA/anti-Ro, anti-SSB/anti-La, anti-RNP) in their blood. may cause problems such as skin rash and heart problems in newborns.
-Lymph nodes: People with primary Sjögren’s syndrome have a 40-fold increased risk of lymphoma compared to the normal population.
– Rarely, neurologically involving nerve endings; may cause burning, numbness, and tingling (peripheral neuropathy) or involve brain tissue.
Diagnosis and Laboratory tests:
First of all, the causes that may cause dry mouth and eyes, drugs and diseases (such as hepatitis C infection, diabetes mellitus, sarcoidosis, radiation to the neck) should be excluded.
-A saliva test is done to show dry mouth. With at least 4 hours of fasting, without salivary stimulant, saliva is accumulated for 15 minutes, dry mouth is 1.5 ml or less.
-To reveal dry eyes; Schirmer test (amount of tears), tear refraction test (quality of tear), and dye tests (to show epithelial damage in the cornea) are performed.
– Complete blood count, liver and kidney function tests, complete urine analysis, blood electrolytes (sodium, potassium) are checked.
-Anti-SSA (aka anti-Ro) and anti-SSB (aka anti-La) antibodies are checked.
-Antibodies to ANA and RF may also be positive.
-Minor salivary gland biopsy (taken from the inner part of the lower lip)
-Sialography
-Saliva gland scintigraphy
-Lung graphy, if necessary lung tomography, Imaging of other organs and systems such as
Sjogren’s Treatment:
– Providing oral hygiene, tooth and gum care (such as mouthwash, use of sensitive-sensitive toothpastes), chewing sugar-free gum to stimulate saliva secretion.
– If it is okay to stimulate salivation, pilocarpine and cevimelin can be used.
-For dry eyes; Artificial tears and gels can be used. If necessary, drops containing cyclosporine can be given by the ophthalmologist.
-Hydroxychloroquine (200mg tb): It has regulatory effects on the immune system. Especially; It is good for fatigue, muscle aches and joint complaints.
– Non-steroidal pain and anti-inflammatory drugs; It can be used for musculoskeletal pain.
-Immunosuppressive treatments are used in the involvement of other organs and systems such as lung and nerve involvement.
Lifestyle and Other Suggestions:
– In case of swelling that may develop in front of the ear or under the chin or on your body, consult your doctor immediately.
-Take care of your oral health and visit your dentist regularly.
– You can moisten your nose using saline sprays.
-It is very important that the environment you are in is humid. For this reason, you can put room humidifiers (household-steam machines) in your home or workplace. Ventilation and air conditioners will increase your dryness. If the artificial tears you use cause itching and redness in the eyes, you can switch to another person by consulting your doctor.
– Drink plenty of fluids and you can circulate sugar-free candy or gum with lemon or different flavors in your mouth that stimulates saliva secretion.
-Intensive moisturizing lotions for skin dryness, immediately after bathing; You can use it while the skin is still moist.
– Get your annual flu shot and long-acting pneumonia vaccine