Lesions of the salivary glands of the type of Sjogren's syndrome. Sjogren's syndrome treatment

Sjogren's syndrome (Sjogren's syndrome) is a chronic autoimmune disease characterized by progressive destruction of the exocrine glands, leading to dry mouth (xerostomia) and eyes (keratoconjunctivitis dry). In patients, an increase in the parotid and salivary glands is revealed.

Primary Sjogren's syndrome associated with HLA-DR3 and manifested by pronounced damage to internal organs is distinguished. The prevalence of primary syndrome is 0.5-1%. Mostly women between the ages of 20 and 30 get sick (the ratio of women to men is 9: 1).

Secondary Sjogren's syndrome is associated with other rheumatic diseases (systemic lupus erythematosus, rheumatoid arthritis, systemic scleroderma) and is associated with HLA-DR4. Secondary syndrome occurs in 30% of patients with rheumatoid arthritis, in 10% of patients with systemic lupus erythematosus, in 1% of patients with systemic scleroderma.

Pathogenesis

There are two mechanisms of tissue damage - lymphocytic infiltration and deposition of immune complexes. In infiltrates of small salivary glands, T-helpers with the properties of memory cells, activated T-and B-lymphocytes, which produce autoantibodies and IL-2, are detected. In the serum, organ-specific autoantibodies are detected, including those to IgM (rheumatoid factor), and to nuclear antigens Ro / SS-A and La / SS-B. T-lymphocytes of infiltrates carry activation markers, including HLA class II, presenting antigens in the epithelial cells of the affected glands.

Symptoms

The leading symptoms of the disease are xerostomia and keratoconjunctivitis. Initially, there is dry mouth, difficulty swallowing dry food and a burning sensation in the mouth, and caries develops quickly. As the disease progresses, dryness spreads to the skin and mucous membranes of the vagina. The second permanent sign is keratoconjunctivitis dry, which is manifested by itching and burning in the eyes, photophobia, dry eyes. When examining the eyes with a slit lamp, pinpoint ulceration of the cornea is visible in the cornea and conjunctiva stained with Bengal pink. Dry spots appear on the cornea after instillation of fluorescein.

Primary Sjogren's syndrome

Approximately 75% of patients with primary Sjogren's syndrome (and some patients with secondary syndrome) have enlarged parotid and other large salivary glands, which secrete cloudy saliva or do not secrete it at all, even when the glands are massaged. MRI reveals the non-hemogenic density of these glands. The function of the salivary glands is examined using sialometry, sialography and scintigraphy. Biopsy reveals lymphocytic infiltration in the mucous membrane of the lips. Many patients are affected by the lungs (pneumonia and interstitial fibrosis), gastrointestinal tract (dysphagia, atrophic gastritis, chronic pancreatitis, hepatitis, biliary cirrhosis). In 40% of patients, kidney damage is found, proceeding in the form of interstitial nephritis with tubular acidosis and sometimes with Fanconi's syndrome. In 25% of patients, vasculitis develops, affecting small and medium vessels and manifested by purple on the skin of the lower extremities, recurrent urticaria, multiple mononeuropathy and CNS damage. Hashimoto is seen relatively often.

Secondary Sjogren's syndrome

The secondary syndrome has clinical manifestations of some other rheumatic disease and symptoms of impaired function of the parotid and lacrimal glands.

Pseudolymphoma

People with Sjogren's syndrome have an increased risk of developing pseudolymphoma, manifested by lymphadenopathy, enlarged parotid glands, or nodules in the lungs. In 10% of patients with systemic manifestations of pseudolymphoma, after a while it is transformed into lymphocytic lymphoma (non-Hodgkin's).

Diagnostics

There are no specific laboratory signs of this disease. Reveal normocytic normochromic anemia, increased ESR, hypergammaglobulinemia, RF, autoantibodies to Ro / SS-A in 70% of patients and to La / SS-B in 40%.

Currently, according to the results of the European Cooperative Study, the diagnostic criteria for Sjogren's syndrome are:

• xerophthalmia for more than 3 months;
• xerostomia for more than 3 months, recurrent or persistent edema of the salivary glands;
• laboratory signs of xerophthalmia - positive results of staining with pink Bengal;
• the presence of at least one infiltrate of ≥50 lymphocytes in the area of ​​histological examination of the salivary gland biopsy with an area of ​​4 mm 2;
• lesions of the salivary glands, detected by their scintigraphy or sialography, a decrease in the rate of salivary secretion (≤15 ml in 15 minutes);
• the presence of autoantibodies to Ro / SS-A and La / SS-B, antinuclear antibodies or rheumatoid factor.

In the presence of any three criteria, the diagnosis of Sjogren's syndrome is presumptive, and four or more are reliable.

The differential diagnosis of Sjogren's syndrome is carried out with HIV infection and sarcoidosis, in which xerostomia, xerophthalmia, or enlargement of the parotid glands develop.

• HIV infection, in contrast to Sjogren's syndrome, mainly affects young men. It does not detect antibodies to Ro / SS-A and La / SS-B. A biopsy of the salivary glands reveals infiltrates from CD8 lymphocytes, and a serological test reveals a positive result for HIV.
• With sarcoidosis, persons of any sex and age get sick, antibodies to Ro / SS-A and La / SS-B are not detected, granules are found in the biopsy specimen of the salivary glands and there is no connection with HLA.

Treatment

Sjogren's syndrome is currently incurable. Treatment is symptomatic, aimed at reducing the dryness of the mucous membranes and preventing caries. With xerostomia, careful oral care should be taken, and saline sprays should be used to moisturize the nasal mucosa. You should avoid taking diuretics, antihypertensive drugs, antidepressants and other drugs that reduce the secretion of the salivary and lacrimal glands. At present, the production of artificial salivary fluid has begun, which relieves xerostomia.

For dry keratoconjunctivitis, moisturizing drops (artificial tear fluid) containing hydroxypropyl methylcellulose are used to moisturize the eyes, 1-2 drops in both eyes as needed, sometimes every 30 minutes. Vidisek eye gel is also used, 1 drop in the eye sac 4-5 times a day and before bedtime. For ulceration of the cornea, use an eye ointment with boric acid and cover the affected eye with a bandage. With xerostomia and keratoconjunctivitis, bromhexine is prescribed orally, 16 mg 3 times a day, and pilocarpine 5 mg 3 times a day.

In severe interstitial pneumonia and pulmonary fibrosis, glomerulonephritis and systemic manifestations caused by vasculitis, glucocorticoids should be prescribed in a dose equivalent to 1 mg / kg / day of prednisolone, if they are ineffective - immunosuppressant cyclophosphamide 3 mg / kg daily or 6 mg / kg through day (course dose 6-10 g). But treatment with cytostatic drugs can contribute to the transformation of pseudolymphoma into lymphoma, therefore they are recommended only for the development of life-threatening conditions. With damage to the central nervous system and multiple mononeuropathy, treatment with glucocorticoids at the indicated dose or NSAIDs (diclofenac, indomethacin, ketoprofen) is carried out.

Forecast

The prognosis in patients with Sjogren's syndrome is relatively favorable.

In primary syndrome, oral hygiene, treatment of xerostomia and keratoconjunctivitis, regular examination of the function of the salivary, lacrimal and thyroid glands and internal organs significantly improve the prognosis of the disease. Psychotherapy is of paramount importance in order to convince the patient that the existing symptoms do not shorten the life span.

In the secondary form of the disease, the symptoms are less pronounced, and the prognosis depends on the manifestations of the underlying disease.

This publication provides general information about Sjogren's syndrome. It describes what Sjogren's syndrome is and what the symptoms are.

You will also find out why and who has this disease and how it is diagnosed and treated.

If you have any further questions after reading this publication, please discuss them with your doctor.

What is Sjogren's Syndrome?

Sjogren's syndrome (Sjogren's disease, Gugereau's syndrome, dry syndrome, or PSS)- autoimmune disease; that is, a disease in which the immune system turns against the body's own cells.

Typically, the immune system works to protect the human body from disease by destroying harmful viruses and bacteria.

In the case of Gugereau's syndrome, cells fighting the disease attack various organs of the patient, primarily the tonsils (tonsils, glands), which produce tears and saliva. Damage to these glands leads to a decrease in the quantity and quality of secretion, which causes symptoms such as: dry eyes and dry mouth.

Sjogren's syndrome is also. Diseases characterized by inflammation (signs include: redness, burning, swelling, or pain) and loss of one or more connective or supporting structures of the body.

They especially affect joints, tendons, ligaments, bones and muscles.

Primary and secondary PSSh

Sjogren's syndrome is classified as either primary or secondary.

The main form occurs in people who do not have other rheumatic disorders.

The secondary form occurs in people who already have another rheumatic disease, most often (RA) or (SLE).

Soon, people with these conditions develop symptoms of dry eyes and.

What are the symptoms of Sjogren's syndrome?

Photos of people with Sjogren's syndrome

Guzhero-Sjogren syndrome can cause many symptoms. The main ones are:

• Dry eyes. The eyes with the syndrome may burn or itch. There is grit in the eyes, blurred vision, or disturbed by bright light, especially fluorescent lighting.
• Dry mouth. If you have a dry mouth (xerostomia), it is difficult to swallow, speak, or eat. Feels like my mouth is full of chalk. Since there is no protective saliva, an infection can develop in the mouth.

Sjogren's syndrome can also affect other parts of the body, causing symptoms such as:

• Joint and muscle pain;
• Constantly ;
• Skin rash;
• Dryness in the vagina;
• Numbness or tingling in the extremities;
• Constant fatigue that interferes with daily life.

Who has dry syndrome?

Sjogren's syndrome can occur in people of any gender and age, but in most cases it occurs in middle-aged women (35-40 years), rarely diagnosed in children.

What causes disease Sjogren

Scientists speculate that Sjogren's disease is caused by a combination of genetic and environmental factors. Several different genes appear to be involved, but scientists don't know which ones are associated with the disease.

Scientists believe the trigger could be a viral or bacterial infection. The possibility is also being explored that the nervous systems play a role in this disease.

How is the disease diagnosed?

Diagnoses Sjogren's syndrome based on the patient's medical history, physical examination, and clinical or laboratory test results.

During diagnosis, your doctor will check for clinical signs of Sjogren's syndrome, such as dry mouth or connective tissue disease.

Because there are many reasons for dry mouth and eyes (medication, other medical conditions, or previous treatments such as radiation), your doctor may require more testing.

Blood tests can detect the presence of antibodies common to PSS, including anti-SSA and anti-SSB antibodies or. A biopsy of the salivary glands and other specialized tests will also help confirm the diagnosis.

Which doctor diagnoses and treats Gugereaux syndrome?

Since the symptoms of Sjogren's syndrome develop gradually and are similar to the symptoms of many other diseases, it can take a long time to diagnose the disease. A person will be helped by a number of doctors who can diagnose and participate in the treatment of the disease. This includes:

• rheumatologists (doctors specializing in diseases of the joints, muscles and bones);
• primary care physician, therapist;
• ophthalmologists (eye specialists);
• otolaryngologists (ear, nose and throat specialists).

Usually, all specialists are coordinated by a rheumatologist during treatment.

How is Sjogren's Syndrome Treated?

Sjogren's syndrome is being successfully treated today.

Therapy can be different depending on the person and which parts of the body are affected.

Dry eye treatment.

There are many treatments that can be taken if a doctor detects dry eyes caused by the syndrome. Here is some of them:

• Eye drops. Available with or without prescription, there are many brands, the product keeps the eyes moist. Some drops contain preservatives that can irritate the retina. Drops without preservatives usually do not bother the eyes (Defislez, Bestoksol, Oksial).
• Ointments. Ointments are thicker than eye drops. Moisturizes and protects eyes for several hours, but blurry vision, therefore, it is most effective during sleep (Oftagel).
• Other treatments. In more severe cases, other treatments may be required, such as a blockage or blockage of the tear ducts, anti-inflammatory drugs, or surgery.

Medicines have side effects. Some side effects can be more serious than others. Ask your doctor or pharmacist for advice before buying medicines.

General Eye Care Tips

• Do not use eye drops that irritate the eyes. If one remedy doesn't work, try another. Eye drops that do not contain preservatives are usually needed for long-term use.
• Practice blinking. When reading, watching TV or a computer, you usually blink less, try to follow this and blink 5-6 times a minute.
• Protect your eyes from drafts and wind.
• Install humidifiers in rooms where you spend the most time, including your bedroom.
• Do not smoke or come into contact with smoke.
• Apply mascara only to the ends of your lashes to avoid getting into your eyes.
• Ask your doctor if there are any medications you are taking that are aggravating dryness.

Treating dry mouth.

There are many remedies for dry mouth. You can try some of them yourself at home. Here are some helpful tips:

• Chewing gum and caramel. If your salivary glands are still producing some saliva, you can stimulate the glands with chewing gum or sucking candy to increase saliva. However, the gum and candy must be sugar-free.
• Water. Consume water or other non-carbonated, sugar-free drinks throughout the day to keep your mouth moist, especially when you eat or speak. Note that drinking plenty of fluids throughout the day will not make your mouth less dry or cause you to urinate more often. You should drink the water in small sips and not too often. Drinking water frequently can reduce or remove the mucus lining in your mouth, increasing the feeling of dryness.
• Other treatments. The doctor may prescribe other treatments, such as medications that stimulate the salivary glands to produce saliva in the mouth.

Treating symptoms in other parts of the body.

If you have an extragranular procedure, meaning a problem that goes beyond the moisture-containing glands of your eyes and mouth, your doctor or appropriate specialist may treat these problems with non-steroidal anti-inflammatory drugs (NSAIDs or immunomodulatory drugs).

A warning: side effects of NSAID use may include:

• stomach problems;
• skin rash;
• high blood pressure;
• fluid retention;
• , kidneys and heart.

The longer a person takes (NSAIDs), the more likely it is that side effects will appear. Many other medications should not be taken while a patient is being treated with NSAIDs. Check with your doctor before taking NSAIDs.

People with Sjogren's syndrome may develop hoarseness when the vocal cords become inflamed as part of an illness or become irritated from a dry throat or cough.

Take a sip of water to prevent further stress on your vocal cords.

Other diseases associated with Sjogren's syndrome

These are the other connective tissues associated with Sjogren's syndrome:

• Polymyositis. Inflammation of the muscles, causing weakness, pain, in some cases, problems with breathing and swallowing. If the skin also becomes inflamed, it is called dermatomyositis.
• (RA). A form of arthritis characterized by severe joint inflammation. The inflammation can damage the surrounding bones (fingers, hands, knees). Rheumatoid arthritis can also damage muscles, blood vessels, and major organs.
• . A disease in which the body builds up too much collagen, a protein commonly found in the skin. The result is thick, tough skin and possibly damage to muscles, joints, and internal organs (esophagus, gastrointestinal tract, lungs, heart, kidneys, and blood vessels).
• Systemic lupus erythematosus (SLE). The disease causes joint and muscle pain, weakness, skin rashes and, in more severe cases, problems with the heart, lungs, kidneys, and nervous system.
.

Sjogren's syndrome is an autoimmune disease that causes systemic damage to connective tissues. The exocrine glands - salivary and lacrimal glands - have to suffer the most from the pathological process. In most cases, the disease is chronic and progressive.

Sjogren's syndrome - what is this disease?

The complex of symptoms of dry syndrome was first noticed by the Swedish ophthalmologist Shegren a little less than a hundred years ago. He found in a large number of his patients, who came to him with complaints of dry eyes, a couple of other identical symptoms: chronic inflammation of the joints and xerostomia - dryness of the oral mucosa. The observation also interested other doctors and scientists. It turned out that this pathology is common and specific treatment is required to combat it.

Sjogren's syndrome - what is it? This chronic autoimmune disease occurs when the immune system malfunctions. The body takes individual cells of its own for foreign and begins to actively produce antibodies to them. Against this background, an inflammatory process develops, which leads to a decrease in the function of the glands of external secretion - as a rule, salivary and lacrimal glands.

Sjogren's syndrome - causes

To say unequivocally why autoimmune diseases develop, medicine cannot yet. Therefore, where the dry Sjogren's syndrome comes from is a mystery. It is known that genetic, immunological, hormonal and some external factors are involved in the development of the disease. In most cases, viruses - cytomegalovirus, Epstein-Barr, herpes - or diseases such as polymyositis, systemic scleroderma, lupus erythematosus, rheumatoid arthritis become the impetus for the development of the disease.

Primary Sjogren's syndrome

There are two main types of disease. But they appear in approximately the same way. Drying of mucous membranes in both cases develops due to lymphocytic infiltration of exocrine glands along the gastrointestinal tract and respiratory tract. If the disease develops as an independent one and nothing preceded its appearance, then this is the primary Sjogren's disease.

Secondary Sjogren's syndrome

As practice shows, in some cases, the disease occurs against the background of other diagnoses. According to statistics, secondary dry syndrome is found in 20 - 25% of patients. It is put when the disease meets the criteria for such problems as rheumatoid arthritis, dermatomyositis, scleroderma and others associated with lesions of connective tissues.

Sjogren's syndrome - symptoms

All manifestations of the disease are usually divided into glandular and extraglandular. The fact that Sjogren's syndrome has spread to the lacrimal glands can be understood by the burning sensation, "sand" in the eyes. Many complain of severe itching of the eyelids. Often the eyes turn red, and a viscous whitish substance accumulates in their corners. As the disease progresses, photophobia arises, the palpebral fissures noticeably narrow, and visual acuity deteriorates. Enlargement of the lacrimal glands is rare.

Typical signs of Sjogren's syndrome, which struck the salivary glands: dry mucous membranes in the mouth, red border, lips. Often, patients develop seizures, and, in addition to salivary glands, some nearby glands also enlarge. At first, the disease manifests itself only during physical exertion or emotional stress. But later the dryness becomes permanent, the lips become covered with crusts that crack, which increases the risk of attaching a fungal infection.

Dryness in the nasopharynx sometimes causes crusts to form in the nose and auditory tubes, which can lead to otitis media and even temporary hearing loss. When the pharynx and vocal cords are very dry, hoarseness and hoarseness appear. And it also happens that a violation of swallowing leads to atrophic gastritis. The diagnosis is manifested by nausea, loss of appetite, heaviness in the epigastric region after eating.

Extraglandular manifestations of the symptom complex of Sjogren's syndrome look like this:

• joint pain;
• stiff muscles and joints in the morning;
• muscle weakness;
• small bloody rash that occurs against the background of vascular lesions;
• neuritis of the facial or trigeminal nerves;
• hemorrhagic rash on the trunk and limbs;
• dryness of the vagina.

Sjogren's syndrome - differential diagnosis

The definition of the disease is mainly based on the presence of xerophthalmia or xerostomia. The latter is diagnosed with sialography, parotid scintigraphy, and salivary gland biopsy. To diagnose xerophthalmia, the Schirmer test is performed. One end of a strip of filtered paper is placed under the lower eyelid and left on for a while. In healthy people, about 15 mm of the strip will get wet after 5 minutes. If Sjogren's syndrome is confirmed, the diagnosis shows that no more than 5 mm is wet.

In differential diagnosis, it is important to remember that SS can develop in parallel with such diagnoses as autoimmune thyroiditis, percinous anemia, and drug disease. The identification of SS-B antibodies significantly contributes to the definition of primary dry syndrome. The most difficult thing to diagnose is Sjogren's disease with rheumatoid arthritis, because the damage to the joints begins long before signs of dryness appear.

Sjogren's syndrome - tests

Diagnosis of the disease involves laboratory tests. When diagnosed with Sjogren's disease, tests show approximately the following results:

1. In the general analysis of blood, accelerated ESR, anemia and a low level of leukocytes are determined.
2. OAM is characterized by the presence of protein.
3. The protein is also elevated in the biochemical blood test. In addition, the study reveals the maximum titers of rheumatoid factor.
4. A special blood test for the presence of antibodies to thyroglobulin in 35% shows an increase in their concentration.
5. Biopsy results of the salivary glands confirm the symptoms of Sjogren's syndrome.

Sjogren's syndrome - treatment

This is a serious problem, but not fatal. If you pay attention to its signs in time and start treatment with the diagnosis of Sjogren's disease, you can live with it, feeling quite comfortable at the same time. The main thing for patients is not to forget about the importance of a healthy lifestyle. This will help to strengthen the immune system, prevent the active development of the pathological process and significantly reduce the risk of complications.

Can Sjogren's Syndrome Be Cured?

Once the diagnosis is confirmed, the patient is given therapeutic advice. Sjogren's syndrome is being successfully treated today, but it is not yet possible to get rid of the disease completely. For this reason, only symptomatic therapy is carried out. The criteria for assessing the quality of treatment is the normalization of the clinical manifestations of the disease. If all therapeutic prescriptions help, laboratory parameters and histological findings improve.

Dry syndrome therapy involves relieving symptoms and, if necessary, combating an underlying autoimmune disease. Before treating Sjogren's syndrome, a diagnosis is required. After, as a rule, the following means are used:

• glucocorticoid Prednisolone (the scheme and dosage is determined individually, depending on the severity of the syndrome and the severity of its symptoms);
• angioprotectors - Parmidin, Solcoseryl;
• immunomodulator Splenin;
• anticoagulant Heparin;
• Contrikal or Trasilol - drugs that interrupt the production of proteolytic enzymes;
• Cytostatics - Chlorbutin, Azathioprine, Cyclophosphamide (generally recommended to be taken together with glucocorticoids).

To get rid of dry mouth, gargle is prescribed. Dry eye syndrome is treated with saline instillation, Gemodez. Overdried bronchi and trachea can be helped by the drug Bromhexine. Applications with Dimexide, Hydrocortisone or Heparin fight against inflammation of the glands. Sometimes dry mouth when diagnosed with Sjogren's syndrome leads to the development of dental diseases. To prevent them, you need to take care of maximum oral hygiene.

Sjogren's disease - treatment with folk remedies

Dry syndrome is a whole complex of symptoms and signs. It is better to fight all of them traditionally. But sometimes, in the case of Sjogren's syndrome, alternative methods, applied in parallel, help to improve the patient's condition. Some patients, for example, note that eye drops from dill and potato juice are much more effective than pharmaceutical tear fluids.

Herbal decoction for gargling

Ingredients:

• chamomile flowers - 1 tbsp;
• sage leaves - 1 tbsp. l .;
• water - 1 glass.

Preparation and application:

1. Mix the herbs and chop lightly.
2. Boil water and pour into the dry mixture.
3. The medicine needs to be infused for 40 minutes.
4. After straining, it is ready to use.

Sjogren's syndrome - prognosis

This disease proceeds without threat to life. But because of it, the quality of life of patients deteriorates markedly. Treatment helps prevent complications and keeps adults working - Sjogren's syndrome is extremely rare in children. If therapy is not started, the disease can develop into a severe form, which, with the addition of secondary infections such as bronchopneumonia, sinusitis or recurrent tracheitis, sometimes leads to disability.

Sjogren's syndrome ("dry syndrome") is manifested by a decrease in the function of the glands of external secretion, as a result of this pathology, there is a pronounced dryness of the skin and mucous membrane of the vagina, trachea, nasopharynx, eyes, oral cavity, there is also a decrease in the secretion of digestive enzymes that the pancreas produces.

Most often, this syndrome is accompanied by a number of autoimmune pathologies of connective tissue - dermatomyositis, scleroderma, and in such cases it is called secondary Sjogren's syndrome. If the pathology develops on its own, then the name sounds like primary Sjogren's syndrome, or Sjogren's disease.

What it is?

Sjogren's syndrome is an autoimmune systemic damage to the connective tissue, manifested by the involvement of the external secretion glands, mainly salivary and lacrimal glands, in the pathological process, and by a chronic progressive course.

Pathomorphology

The main morphological feature is the infiltration of the external secretion glands by lymph and plasma cells. First of all, the lacrimal and salivary glands suffer, a little less often - the glands of the bronchi, digestive tract and vagina.

1. Both large and small glands are affected. At first, at an early stage of the disease, only small ducts are involved in the process; as it progresses, its infiltrate spreads further, to the gland tissue itself, as a result of which the glandular tissue atrophies and is replaced by connective tissue. In some cases, the infiltrates described above occur not only in the exocrine (external secretion) glands, but also in other organs and systems of the body, in particular, in the muscles, lungs and kidneys. This ultimately leads to dysfunction of the affected organ.
2. In 30-40% of patients in the material taken by biopsy of the salivary glands, metaplasia (modification) of the cells lining the ducts is determined: myoepithelial islets appear.

The lobules of the affected glands in a number of patients are destroyed, while in others the lobular structure is preserved. The glands are either enlarged or within normal limits.

Interestingly, even in the absence of a vivid clinical symptomatology of Sjogren's syndrome, a patient with any connective tissue disease is likely to have histological signs of inflammation of the salivary glands.

Reasons for development

The causes of Sjogren's syndrome have not yet been fully established. Among the most probable is the theory of the pathological reaction of the body's immune system. Such a reaction develops in response to damage to the cells of the external glands by a retrovirus, in particular, it is the Epstein-Barr virus, VI herpes virus, cytomegalovirus, human immunodeficiency virus. Despite the significant similarity of immunological disorders with changes in the organism affected by the virus, direct evidence of the role of the virus as a cause of the development of pathology has not been obtained.

The viruses themselves and the epithelial cells of the glands, altered by their influence, are perceived by the immune system as antigens (foreign agents). The immune system produces antibodies against these cells and gradually causes destruction of the gland tissue. The disorder is often hereditary or familial, especially in twins, suggesting a genetic predisposition.

Thus, it is assumed that a combination of many factors is important in the mechanism of development and occurrence of pathology:

• stress reaction of the body, which occurs as a result of the immune response;
• immune regulation with the participation of sex hormones, as evidenced by the rare incidence among people under 20 years old, while among children, girls most often get sick;
• immune control with T-lymphocytes;
• viral;
• genetic.

There are two types of Sjogren's syndrome: primary - the symptoms of the disease are the first manifestations of it, and secondary, when the symptoms appear in patients suffering from other rheumatic diseases, such as scleroderma, rheumatoid arthritis or systemic lupus erythematosus. Primary and secondary variants of the syndrome occur with approximately the same frequency. This sometimes makes it difficult to make an accurate diagnosis. Sjogren's syndrome is quite common: in the UK, for example, there are about half a million patients. The most commonly affected women are between the ages of 40 and 60, while only one out of 13 patients is male.

Symptoms of Sjogren's Syndrome

The symptoms of the disease are divided into two groups:

1. Glandular manifestations, in which the epithelial glands are affected and their functions are impaired.
2. Extra-mucous manifestations. The symptomatology of this group is very different, due to the defeat of various human organs.

Glandular symptoms, the following can be distinguished:

1. Pathology of the lacrimal glands. In this case, there are unpleasant and painful sensations in the eyes (burning, the eyes seem to start to cut, the sensation of sand in them), while there is a characteristic itching near the eyes, redness. As a result, vision decreases, the occurrence of punctate hemorrhages, edema, sensitivity to light, eye pain.
2. Pathology of the salivary glands. The manifestation of inflammation, mainly of the parotid glands, which are enlarged, sometimes with the manifestation of painful sensations. Also, there is dryness of the mucous membrane of the mouth, swallowing food is difficult, often patients have to drink water with food, due to the problem of swallowing. In this case, the mucous membrane of the mouth takes on a bright pinkish tint.
3. Dryness of the respiratory tract, which leads to inflammation of the bronchi, trachea, lungs.
4. Skin disorders resulting in dry skin.
5. Damage to the nasal mucosa. The onset of dryness in the nose, covering with an intranasal crust, as a result of which inflammation develops.

Extra-mucous manifestations are characterized by the following symptoms:

1. The onset of tracheobronchitis, accompanied by cough, shortness of breath. It is not uncommon for a patient to be diagnosed with pneumonia or pulmonary fibrosis during examination.
2. Swollen lymph nodes.
3. Damage to the peripheral nervous system. In this case, pain begins to appear (tingling, burning).
4. Fever
5. Joint and muscle pain.
6. Damage to the thyroid gland. It is a rare disorder in which allergic reactions to various foods, medications and other drugs begin to appear.
7. Enlarged lymph nodes (submandibular, cervical, occipital). Enlargement of the liver and spleen is not uncommon.
8. Vascular inflammation, which occurs in the case of atherosclerosis of the lower extremities, due to circulatory disorders. If the functioning of the vessels is impaired, skin diseases (rash) appear, which are accompanied by itching, burning sensation and fever.

Often, against the background of the pathology described by us, patients develop an increased individual sensitivity to certain drugs, in particular, to non-steroidal anti-inflammatory drugs, some antibiotics (penicillin), drugs of the so-called basic therapy, cytostatics.

Diagnostics

Burning eyes and dry mouth may not always mean that a person has this syndrome. It is possible to diagnose Sjogren's syndrome only if there is an inflammatory lesion of the glands. However, there are cases when various metabolic diseases can lead to a similar result, in which the secretion of saliva is significantly reduced (most often it is diabetes mellitus).

As a result, in people of old age, the functions of the salivary and lacrimal glands prematurely decrease. These forms of dry eyes and mouth have nothing to do with Sjogren's syndrome. Also, to diagnose this syndrome, a tissue study is carried out. For this type of examination, small fragments of the oral mucosa are taken, which are examined using a microscope. Thus, the defeat of the mucous glands is established.

Complications

Common consequences of Sjogren's syndrome:

• lymphomas (neoplasms that affect the blood, lymph nodes);
• vasculitis (an inflammatory process in the vessels, which can occur everywhere);
• accession of a secondary infection;
• the development of oncological diseases;
• inhibition of blood formation, reduction in the blood of leukocytes, erythrocytes and / or platelets.

If the patient does not take any measures to treat Sjogren's disease for a long time, or if he was prescribed the wrong therapy, the pathology progresses, leading to serious disruptions in the functioning of organs and systems.

Treatment for Sjogren's syndrome

In the presence of Sjogren's syndrome, treatment is carried out depending on the stage of the disease and the presence of systemic manifestations.

In order to stimulate the function of the glands, the following is carried out:

• drip introduction of kontrikal.
• subcutaneous administration of galantamine.
• with a general strengthening purpose, courses of vitamin therapy are carried out.
• As a symptomatic treatment, “artificial tears” (drops in the eyes) are prescribed - with low viscosity - Lakrisify (200-250 rubles), Natural tear (250 rubles), medium viscosity Lacrisin, high viscosity Oftagel 180 rubles, Vidisik 200 rubles, Lakropos 150 rubles ...

At the initial stages, in the absence of damage to other systems and unexpressed laboratory changes, long courses of glucocorticosteroids (prednisone, dexamethasone) in small doses are prescribed.

If the symptoms and laboratory parameters are significantly pronounced, but there are no systemic manifestations, cytostatic immunosuppressive drugs are added to corticosteroids - cyclophosphamide, chlorbutin, azathioprine. Supportive therapy is carried out with the same means for several years.

1. In the presence of symptoms of systemic damage, regardless of the stage of the disease, high doses of corticosteroids and immunosuppressants are immediately prescribed for several days with a gradual transfer to maintenance doses.
2. With generalized polyneuritis, vasculitis, kidney damage and other severe manifestations of the disease, such methods are added to the above treatment, extracorporeal treatment - plasmapheresis, hemosorption, plasma ultrafiltration.
3. The rest of the drugs are prescribed depending on the complications and concomitant diseases - cholecystitis, gastritis, pneumonia, endocervicitis, etc.

In certain cases, it is necessary to adhere to dietary nutrition and limit physical activity.

Prevention

To prevent a violation, you must follow all the instructions of the attending physician to prevent exacerbations. You also need to follow a few simple rules:

• limit the load on the organs of vision;
• if the syndrome arose as a result of another disease, the cause of the occurrence must be treated.
• avoid stressful situations.

Forecast

Sjogren's syndrome can damage vital organs with a transition to a stable state, gradual progression, or, conversely, long-term remission. This behavior is common in other autoimmune diseases.

• Some patients may have mild symptoms of dry eyes and mouth, while others develop serious complications. Some patients are fully helped by symptomatic treatment, while others have to constantly struggle with blurred vision, constant eye discomfort, often recurrent infections of the oral cavity, edema of the parotid salivary gland, difficulty chewing and swallowing. Constant loss of strength and joint pain seriously reduce the quality of life. In some patients, the kidneys are involved in the pathological process - glomerulonephritis, leading to proteinuria, impaired renal concentrating ability and distal renal tubular acidosis.
• People with Sjogren's syndrome have a higher risk of developing non-Hodgkin's lymphoma compared to healthy people and people with other autoimmune diseases. About 5% of patients develop some form of lymphoma.

In addition, it was found that the children of women with Sjogren's syndrome during pregnancy have a higher risk of developing neonatal lupus erythematosus with congenital heart block.

Sjogren's syndrome or "dry syndrome" is an autoimmune disease that can develop on its own or as a complication of chronic pathology. Scientists have found that about 1% of the world's population suffers from this disease.

An interesting fact is that women between the ages of 20 and 50 are more likely to get sick. Experts around the world are still working on the study of the causes of the development of the syndrome.

Treatment of the disease is quite complex and lengthy and depends on the severity of the patient's condition.

Causes of the pathological condition

Sjogren's syndrome is a pathological condition in which the glands of external secretion are affected. It manifests itself as dryness of the mucous membranes of the mouth, nasopharynx, vagina, trachea, eyes. And also the production of digestive enzymes that the pancreas produces is reduced. Scientists have not figured out the exact reasons, however, presumably the disease develops due to the following factors.

• The body's immune response to retrovirus damage (HIV, cytomegalovirus, herpes virus).
• A genetic predisposition that often occurs in twins.

• Some researchers have suggested a viral etiology.
• Stressful situation due to the body's reaction to the intervention of retroviruses.
• Nervous and physical fatigue.

Often, pathology accompanies some systemic diseases: lupus erythematosus, scleroderma, sarcoidosis and others.

Symptoms of the disease

When a disease is a complication of an existing chronic pathology, they speak of a secondary syndrome, if it is an independent disease, it is called Sjogren's disease.

The symptoms of Sjogren's disease are varied and can affect all organs and systems. They are divided into glandular and non-glandular. The following features are glandular.

• Xerostomia - dryness of the mucous membranes of the mouth, which develops as a result of a decrease in the productivity of the salivary glands. This makes speech difficult and can lead to dysphagia, impaired swallowing.
• Xerophthalmia, or dry eyes, is also a result of decreased performance of the lacrimal glands.
• Inflammatory processes in the ear glands, hearing impairment, inflammation of the Eustachian tube.

Development of stomatitis, caries, seizure in the corners of the mouth, leading to cracks. If you do not seek medical help in time, caries provokes complete tooth decay.

Crashes and itching in the eyes, the feeling that sand has been poured in them and a foreign body is present. The eyelids become hyperemic, edematous, whitish plaque accumulates in the corners of the eyes, and photophobia develops. One of the symptoms is blurred vision and ulceration of the cornea.

Mucous membranes organs of the respiratory system dry up, crusts form in the nose, which are difficult to remove, sinusitis, tracheitis, pneumonia, tracheobronchitis develop. In some cases, chronic parotitis occurs.

• Decreased work of sweat glands.
• From the side of the external genital organs, there is a burning sensation and soreness.
• Inflammatory processes of the bile ducts are manifested by dyskenesia of the biliary tract.
• Malfunctions of organs gastrointestinal tract: indigestion, pain in the stomach and intestines, nausea, vomiting.

Today, there are also symptoms of the disease that do not belong to disturbances in the functioning of the glands of external secretion, but significantly worsen the patient's condition. These are the so-called extraglandular signs.

• Hyperthermia and muscle soreness.
• Inflammatory processes in the joints, up to the development of rheumatoid arthritis.
• Violation of blood circulation, manifested by the appearance of ulcers, bruises and long-term non-healing wounds.
• Pathological processes in the small intestine, leading to the death of one or more of its segments.
• Renal failure, glomerulonephritis and urolithiasis due to kidney damage.
• Due to insufficient blood supply to the fingers, Raynaud's syndrome can develop.
• Swollen lymph nodes and spleen

• Neurological disorders: polyneuropathy, neuritis of the trigeminal and facial nerve.
• The onset of drug allergies.
• Chronic fatigue syndrome and decreased performance.
• Disruption of the thyroid gland.
• Decreased immunity leads to the development of pulmonary fibrosis and pleurisy.

In advanced cases, autoimmune failure can provoke the development of cancer.

Diagnosis of Sjogren's disease

First of all, the specialist conducts an objective examination and, on its basis, prescribes additional methods to clarify the diagnosis. A mandatory item is a biochemical and immunological blood test. A biopsy of the salivary gland is also prescribed.

Other additional methods are salivary gland scintigraphy and parotid sialography. To stimulate lacrimation, ammonia can be used.

Drug treatment

Treatment options depend on the severity of the patient's condition and the stage of the disease.

• To stimulate the lacrimal glands, subcutaneous injections of galantamine solution and drip infusions of contrikal are prescribed.
• To eliminate the symptom of dryness of the cornea, special drops are prescribed that replace a human tear: Lakrisifi, Natural Tear, Lakrisin, Vidisik, Lakropos.

As part of the complex treatment, anticoagulants and angioprotectors are prescribed. The appearance of neurological disorders and pathologies of the kidneys is the reason for the appointment of additional methods: plasmapheresis, plasma ultrafiltration. Some cases of the disease require the appointment of a special diet and restriction of physical activity.

Symptomatic treatment includes rinsing the mouth to reduce dryness, instilling the eyes, taking bromhexine to eliminate dryness of the mucous membranes of the trachea and bronchi, lubricating the external genital organs with special creams and gels, and softening the crusts in the nose with various aerosols. Inflammation of the salivary glands is treated with applications with a mixture of Dimexide and hydrocortisone.

An obligatory point of therapy is the intake of multivitamin complexes to strengthen the immune system and increase the body's defenses.

In the initial stages of the disease, glucocorticosteroids are used, for example, Prednisolone or Dexamethasone. Their use is permitted in the absence of pathologies from organs and systems, at the initial stage of the disease.

When drastic changes are noted in laboratory parameters, but there are no systemic disorders, cytostatics are added to hormonal drugs: Aziotropin, Cyclophosphamide and others. The first signs of systemic disorders require the use of corticosteroids in combination with immunosuppressants. The dosage is reduced after a few days and the patient is transferred to the minimum maintenance dose.

All stages of treatment are carried out under the supervision of a specialist who determines the need for the appointment of a particular remedy.

Folk remedies

In folk medicine, there are several recipes for the treatment of Sjogren's syndrome. They are not able to cope with the cause of the pathology, but they significantly alleviate the patient's condition.

1. An oil solution of Chlorophyllipt will help to successfully cope with the symptoms of damage to the nasal mucosa.
2. Gargles with herbal infusions and decoctions of chamomile, sage, coltsfoot, yarrow. It is necessary to infuse herbs for 40 minutes, strain and use for rinsing several times a day.
3. You can improve the condition of the mucous membranes of the eyes with the help of compresses with potato or dill juice. The duration of the procedure is at least 20 minutes.
4. To speed up the healing of cracks in the corners of the mouth will help lubricate them with sea buckthorn oil or rosehip oil.

It is allowed to use folk remedies only after the approval of the attending physician.

Complications of the disease

Untimely access to a specialist leads to complications that are difficult to treat.

• Neoplasms that affect the hematopoietic system and lymph nodes - lymphomas.
• Widespread vasculitis.
• Development of a secondary infection.
• Malignant oncological diseases.
• Inhibition of the formation of blood cells.

The first signs of Sjogren's syndrome require immediate medical attention. This will help start treatment at an early stage of the pathology and eliminate the risk of complications.

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