Sjögren’s syndrome – causes, symptoms, treatment. Living with Sjögren’s syndrome

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Sjögren’s syndrome is an autoimmune disease in which there are defects in the lacrimal gland and salivary glands. It is with them that the first symptoms of the disease are associated, i.e. dry conjunctiva and associated inflammation and dry mouth.

Sjögren’s syndrome – causes

In the course of Sjögren’s syndrome, autoantibodies are produced in the body, which damage the salivary and tear glands and disrupt their function. The effect of autoantibodies is the lack of saliva and tears, associated with unnatural dryness of the mucous membranes. There are two types of Sjögren’s syndrome:

1. primary;
2. secondary: accompanies other immune disorders (e.g. rheumatoid arthritis, visceral lupus);

See also: Impairment of tear secretion

Sjögren’s syndrome – risk factors

There are factors that can increase your likelihood of having Sjögren’s syndrome, including:

1. age – Sjögren’s syndrome usually affects people over the age of 40, but younger adults and children can also get it;
2. sex – women are 10 times more likely to develop Sjogren’s syndrome than men;
3. other autoimmune problems – Almost half of all people with Sjogren’s syndrome also have another autoimmune disease, such as lupus and rheumatoid arthritis.

Sjögren’s syndrome – symptoms

The symptoms associated with Sjögren’s syndrome are closely related to its cause, which is dryness of the lacrimal glands and salivary glands. Patients complain of dry conjunctiva, burning eyes, excessive sensitivity of the eye to the wind. There may be irritation and redness. Objectively, the tear shortage is confirmed by performing the so-called Schirmer testwhich measures the length of a tear-soaked length of tissue paper placed in the corner of the patient’s eye.

Another symptom of Sjögren’s syndrome is damaged and enlarged parotid or submandibular glands. Their defect is associated with the difficulty of chewing and swallowing food, talking, tasting food. The Saxon test is the test that confirms the lack of salivain which the patient is asked to chew a sterile gauze pad and the extent to which it is soaked with saliva is checked.

When it comes to examining changes in the salivary glands, there is a whole spectrum of possible imaging tests, performed depending on their availability in a given center (sialography, scintigraphy, ultrasound or magnetic resonance imaging). It is also possible to perform a puncture (biopsy) of the affected salivary gland and collect its fragment for examination under the microscope (the so-called histopathological examination), which confirms the presence of abnormal infiltrates from the immune system cells – lymphocytes.

See also: Do your eyes hurt? This could be a symptom of COVID-19

Sjögren’s syndrome – symptoms not related to the eyes

While the main symptoms of Sjögren’s syndrome are associated with dry eyes and mouth, it is important to remember that it is a generalized disease and that inflammation is ongoing throughout the body. Hence, patients often report symptoms such as weakness, loss of appetite, difficulty sleeping, and sometimes symptoms of depression may also occur. Joint pains are frequent, patients also complain of freezing and blue blue of the backs of the hands (the so-called Raynaud’s phenomenon).

Much less often, lymphadenopathy, skin involvement (erythema, vasculitis), damage to the lungs, kidneys, pancreas, thyroid gland and the nervous system may occur. In women, symptoms of vaginal dryness are common, making intercourse difficult and causing pain during intercourse.

See also: Why do some women feel pain during intercourse?

Diagnosing Sjögren’s syndrome

The diagnosis of the disease is made on the basis of typical symptoms, the autoimmune cause of which is confirmed either by histopathological examination of the salivary glands or by the presence of circulating antibodies characteristic of the disease in the blood. However, since in 60% of cases Sjögren’s syndrome accompanies other autoimmune diseases, it is also necessary to perform specialized tests to exclude or confirm the existence of other symptoms and abnormalities.

It is important to monitor whether internal organs are damaged in the course of the disease. It is important to exclude diseases such as infection with hepatitis C virus and HIV. Another problem is the need to periodically control patients with diagnosed disease, because it is known that Sjögren’s syndrome, according to some sources, is associated with up to 40 times higher risk of developing lymphomas. However, both the symptoms of Sjögren’s syndrome and the lymphomas that develop due to it, usually progress slowly and secretly, which makes early cancer diagnosis difficult and significantly hinders its effective treatment.

The tests performed in the diagnosis of Sjögren’s syndrome include the following.

1. Blood tests: People with Sjögren’s syndrome often have high levels of inflammation in their blood, which can be measured with blood tests, such as the red blood cell sedimentation rate (ESR). Two autoantibodies, called anti-Ro and anti-La, are important in diagnosis and can be found in blood tests. About two-thirds of people with primary Sjogren’s syndrome have anti-Ro or anti-La antibodies, or both. The presence of one or more of these autoantibodies in a person with dry eyes or dry mouth strongly suggests that they have this condition. However, not everyone with this condition has these autoantibodies, so your doctor may need to do other tests.
2. Biopsy: Your doctor may remove tissue or cells from the salivary gland or the inside of the lip. The collected sample is sent to the laboratory to check for signs of inflammation. It can also be used to check if you are at risk of developing lymphoma.
3. Eye exam: A specialist, such as an ophthalmologist, can measure tear production. During an eye exam, your doctor will examine the cornea, the transparent part of the eye, for dryness.
4. Imaging tests: These include sialometry, which measures the amount of saliva produced using X-rays that can see the dye injected into the salivary glands. There is also salivary gland scintigraphy, a way to track how long it takes for a radioactive isotope to travel from the injection point in the vein to the salivary glands. In addition, ultrasound and magnetic resonance imaging (MRI) can be used. The glands are usually gray on ultrasound images, but in people with Sjögren’s syndrome, the scans show round black glands. MRI is used when the diagnosis is uncertain, or it is believed that you may have another problem, such as lymphoma.

Sjögren’s syndrome – treatment

Treatment of Sjögren’s syndrome is based on relieving discomfort resulting from dry eyes and mouth. The causal treatment of the disease is not possible.

Treatments for Sjögren’s syndrome symptoms related to the eyes

If a person’s eyes are dry and sore, they may use lubricating eye drops to keep the eyes hydrated and reduce friction. You can buy them yourself in pharmacies. You may need to talk to your doctor or other health care professional and try a few different types of eye drops before finding the right one for you.

For very dry eyes, you may need to use ointments, gels or sprays, as well as eye drops. Your doctor may give you eye drops containing cyclosporine or steroids if your eyes are inflamed. Your doctor may also give you mucolytic eye drops if mucus has made your eyes sticky. Paraffin-based ointments can be used overnight to help keep your eyes dry when you wake up in the morning.

If a person needs to use eye drops more than three times a day, it is a good idea to use a preparation that does not contain preservatives, as regular use of eye drops containing preservatives can increase dryness and sensitivity. If you have corneal ulceration or infections, talk to a specialist before using any products.

In some cases, the ophthalmologist may suggest closing the tear points. This is where tiny plugs are inserted into the channels at the inner corners of the eyelids. This reduces the ability of the tubes to drain tears or eye drops from the surface of the eye. Usually temporary plugs are tried first, and if they are helpful, more durable plugs can be inserted. If the eyes remain very dry, the plugs can be heat sealed in a procedure called cauterization.

Treatments for the symptoms of Sjögren’s syndrome related to the mouth and throat

If you have problems with dry mouth, you should see your dentist every three to six months so that they can monitor and treat their symptoms.

You may find that “artificial saliva” preparations, lozenges, sugar-free gum or gels reduce dry mouth. Sprays are also good, but some are acidic and can increase tooth decay, so make sure you use fluoride-containing sprays. Antifungal treatment can also help if your mouth is very dry, which causes other problems such as fungal infections and a dry cough. Chlorhexidine mouth rinses can help with oral hygiene, but they should be used briefly, only occasionally, as they may discolor your teeth.

You can also buy toothpaste or fluoride foams to help reduce tooth decay. Patients should try to use non-alcoholic products, as alcohol-based products can cause additional dryness of the mouth. Most of the above products should be available at your pharmacy or supermarket.

If your salivary glands are causing pain in the sides of your face, talk to your doctor as it may be caused by an infection. If it’s not an infection, your doctor may prescribe a short course of a steroid called prednisolone or use a steroid injection to reduce inflammation.

See also: Polish scientists have developed artificial saliva

Other treatments for Sjögren’s syndrome

Medications such as nifedipine, which open blood vessels, may be prescribed if the patient has Raynaud’s phenomenon. Antispasmodics, such as mebeverine, may be helpful in treating stomach pain and bowel problems.

If a woman has a problem with vaginal dryness, she can use hormone-free creams to improve the condition. It is good for her to ask her doctor for more information on this. Hormone replacement therapy (HRT) can be used for more severe menopausal symptoms, but it’s important to discuss your risk of side effects with your doctor.

It should also be remembered that pregnant women with anti-Ro antibodies are at risk of developing a heart block in their baby. These patients require specialist care during pregnancy and appropriate preventive treatment.

Medicines used to treat Sjögren’s syndrome

There are also many medications that can be used to treat Sjögren’s syndrome.

Pilocarpine for Sjögren’s syndrome

Pilocarpine tablets can increase the amount of saliva and tears that salivary glands produce. It can also help reduce dryness in other areas of the body, such as the throat and vagina.

Some people experience side effects, but these usually go away with time. They can be:

1. sweating;
2. headaches;
3. urinating more often.

It may be helpful to start using the preparation at a low dose and gradually increase it as the side effects improve.

Painkillers and NSAIDs for Sjögren’s syndrome

Painkillers and non-steroidal anti-inflammatory drugs (NSAIDs) can be used to treat pain and joint pain. They can be purchased over the counter at supermarkets and pharmacies, but your doctor can prescribe stronger versions if you need them.

NSAIDs help reduce inflammation and control pain, swelling, and stiffness. Common examples are ibuprofen, naproxen and diclofenac. Pain relievers such as acetaminophen can also be used to treat severe headaches along with anti-migraine medications.

Steroids for Sjögren’s syndrome

Steroids, also known as corticosteroids, can be used to treat some symptoms but will not change the condition itself. If the affected joints are inflamed, a steroid injection or a short course of steroid tablets may be suggested as the patient will feel better immediately. If his condition is more severe, he may need to take steroids for a long time.

DMARDs for Sjögren’s syndrome

Disease-modifying antirheumatic drugs (DMARDs) reduce the activity of the immune system. They are sometimes used to treat long-term symptoms of the disease or are given to people with more severe symptoms. These medications are not usually used if the person has only mouth and eye symptoms, but may be given if there are joint problems or organ complications.

Hydroxychloroquine is a DMARD that is usually used to treat joint pain, pain, and fatigue. Other DMARDs that can be used to treat Sjögren’s syndrome include:

1. cyclophosphamide;
2. cyclosporine;
3. azatiopryna;
4. methotrexate;
5. mykofenolan.

Another drug, such as colchicine, may be given. Tacrolimus is another DMARD that can be used to treat skin problems when hydroxychloroquine is not helping. Rituximab is a type of DMARD that targets the B cells of the immune system. It is given to people who have other complications of Sjögren’s syndrome, such as interstitial lung disease, idiopathic thrombocytopenic purpura, or lymphoma. It is also used when steroids and other DMARDs are not working.

See also: Hydroxychloroquine and chloroquine. What about the side effects of drugs tested to treat COVID-19?

Sjögren’s syndrome – Relief of symptoms

There are many things you can do to help relieve your symptoms.

1. Wear sunglasses or glasses with side shields to help keep your eyes moist.
2. Keep the eyelids and the eye area clean to increase the amount of substance that leaves the eyelid glands.
3. Avoid smoky or windy places as this can dry out your eyes.
4. Use humidifiers, plants, or bowls of water to increase humidity and keep indoor air moist.
5. Have regular visits to the optician to check the condition of your eyes.
6. Take regular breaks from the screens – avoid using the computer, telephone or TV set for extended periods of time.
7. Visit your dentist for routine oral checkups. 
8. If the sick person smokes, he or she should try to quit.
9. Avoid using strong soaps if you have dry, itchy skin. Water-based creams and emollients can help.
10. Apply sunscreen with SPF 15 or higher if your skin is sensitive to the sun or if you have a rash.
11. Wear warm gloves if you develop Raynaud’s phenomenon. Exercise will help improve circulation.

See also: Which sunglasses to choose? Important advice from an ophthalmologist

Sjögren’s syndrome and exercises

Exercise is really important in Sjögren’s syndrome, especially if the person has symptoms in the joints.

A physical therapist can advise you on what exercises to do and how to gradually increase your activity levels. Exercise can also help with extreme fatigue, difficulty concentrating, joint stiffness, and symptoms of depression.

See also: Physiotherapy – functions, types. Who Should Use Physiotherapy? [WE EXPLAIN]

Sjögren’s syndrome and diet and nutrition

There is no specific diet recommended for people with Sjögren’s syndrome. However, if you have abdominal pain or gut problems, increasing the amount of fiber in your diet should help. The physician may refer the patient to a dietitian who is trained in giving dietary advice and can provide support and suggestions for alleviating any abdominal problems.

Cutting back on sugary foods and drinks will help reduce any dental problems. Maintaining a healthy weight can also be helpful, and this will also reduce the strain on your joints. Be aware that alcohol, tea, coffee, and other caffeinated drinks can dehydrate you. Regularly sipping water or sucking on ice cubes can help relieve a dry mouth.

Sjögren’s syndrome and complementary treatments

There are no studies showing that adjuvant therapy can improve the symptoms of Sjögren’s syndrome. Most complementary therapies generally do not cause serious side effects in people with this condition, but you should always discuss them with your doctor before starting treatment.

There are some risks associated with certain treatments and it’s important to go to a legally registered therapist. If a person decides to try therapies or supplements, they should carefully consider how they work and whether they notice any improvement before continuing.

Sjögren’s syndrome and the support of other people with this condition

Joining a local support group can be a good way to meet others and get useful tips on managing your condition. You can also find useful tips on online forums through related organizations.

Living with Sjögren’s syndrome

Sjögren’s syndrome and work

Work can provide a sense of purpose, help strengthen social ties, and help financially. Some people find that continuing to work can actually help reduce symptoms such as fatigue and clouding of consciousness.

Sjögren’s syndrome and coping with fatigue

Fatigue is a feeling of weariness or exhaustion. It affects people in different ways, but it can make it difficult to carry out daily activities. The sick person may feel that his limbs are heavy and as if he has no energy.

It’s a good idea to talk to your rheumatism team about treatment, as fatigue can be a side effect of some medications. It can also be due to activity and inflammation, so doctors may want to look at the dose and type of medications you’re taking.

It’s a good idea to follow these handy tips to help reduce fatigue.

1. Increase the amount of physical activity you do to increase your energy levels. Start slowly, even after just 5 or 10 minutes of walking, can make a difference.
2. Eat a balanced diet to make sure you are providing your body with all the nutrients it needs to maintain high energy levels.
3. Introduce relaxation techniques into your daily life to reduce feelings of stress. Listening to music, spending time with friends, and exercising can help.
4. Talk to your doctor, rheumatologist, or friends in low mood as it may be related to fatigue. Talking has been shown to help improve low mood and fatigue.
5. Plan and prioritize the things you need to do and avoid leaving all the demanding tasks at once. Set your pace by breaking down tasks into smaller ones and taking regular breaks.

Sjögren’s syndrome and sex, fertility and pregnancy

Women with Sjögren’s syndrome can sometimes have a dry vagina problem, which can make it painful during sexual intercourse. Lubricants and creams made from the female sex hormone estrogen can help. Medicines for infections such as thrush are available from pharmacies.

Sjögrenanie’s syndrome affects male and female fertility. However, women with anti-Ro or anti-La antibodies may have a higher risk of miscarriage. Your doctor may suggest taking low doses of aspirin in the early stages of pregnancy to reduce this risk.

Usually there are no problems during or after pregnancy, and the expectant mother may be able to continue some therapies, such as hydroxychloroquine, during pregnancy. However, a very small number of women can pass anti-Ro or anti-La antibodies to their baby during pregnancy. Your baby may then develop symptoms such as a rash and abnormal blood tests after birth.

These will disappear when the mother’s antibodies are lost from the baby’s blood. This may take from several weeks to several months. In some cases, antibodies affect the baby’s heart causing it to beat slowly. If the mother has these antibodies, she should tell her obstetrician, as the baby’s heartbeat will require additional monitoring in the womb.

See also: Fertile days calendar. How to calculate fertile and infertile days?

Sjögren’s syndrome – complications

The vast majority of patients with Sjögren’s syndrome remain very healthy, without serious complications. Patients should know that they face an increased risk of infection around the eyes (photosensitivity, blurred vision, and corneal damage) and an increased risk of dental problems due to a long-term lack of tears and saliva (caries). People with Sjögren’s syndrome are also much more likely to develop thrush, a thrush in the mouth.

Rarely, patients may develop complications related to inflammation elsewhere in the body.

1. Lung problems that may mimic pneumonia;
2. Abnormal liver and kidney function tests;
3. Skin rash associated with inflammation of the small blood vessels;
4. Neurological problems causing weakness, numbness, tingling and burning sensation in the hands and feet (peripheral neuropathy).

In a small number of people, Sjögren’s syndrome may be associated with lymphoma, a cancer of the lymph nodes, so patients should visit their doctor regularly for general screening tests. They should also pay close attention to any abnormal swelling of the glands around the face or neck, armpits, or in the groin area.

If you have Sjögren’s syndrome, you may notice your hair loss, and this could be due to your illness. There is a condition known as frontal fibrosing alopecia (FFA) that is more common in people (mostly women) with autoimmune diseases. This condition causes hair to fall off slowly in the front hairline and sometimes in the eyebrows. However, hair loss can be triggered by a number of factors, including stressful life events and medications. It is worth asking a dermatologist about problems with hair loss.