Sjögren's syndrome - all the features of successful treatment. Sjögren's disease - causes, classification, diagnosis, treatment with medications and folk remedies Sjögren's disease symptoms treatment for what

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Sjögren's syndrome ("siggy syndrome") is an autoimmune lesion of the exocrine (primarily lacrimal and salivary) glands, accompanied by their hypofunction and usually combined with systemic immuno-inflammatory diseases. This definition, which seems to us the most accurate from a scientific point of view, quite fully characterizes the essence of this pathology.

The diagnostic triad of K. Bloch et al. is popular among clinicians. (1965):

1) dry keratoconjunctivitis with enlarged or normal size of the lacrimal glands;

2) xerostomia with enlarged or normal size of the salivary glands; 3) the presence of a systemic connective tissue disease (more often rheumatoid arthritis (RA), less often systemic lupus erythematosus (SCR), systemic scleroderma (SSD), even less often - polyarteritis nodosa or dermatomyositis).

Most authors consider two of these three manifestations sufficient and necessary for the diagnosis of Sjögren's syndrome. The combination of sicca with RA or another connective tissue disease is considered secondary Sjögren's syndrome, and the presence of only keratoconjunctivitis sicca and xerostomia is considered primary Sjögren's syndrome (or "Sjögren's disease", in less accepted terminology). The disease occurs in representatives of all races and at different ages (including children). More than 90% of all patients are middle-aged and elderly women.

Etiology and pathogenesis

Main histomorphological feature- lymphocytic and plasma cell infiltration of salivary, lacrimal and other exocrine glands - bronchial, gastrointestinal, vaginal. Typically, damage occurs to both large salivary glands (parotid, submandibular) and small ones located in the mucous membrane of the gums and palate. At the first stages, the infiltrate is located around small intralobular ducts; later it spreads throughout the parenchyma of the gland, sometimes accompanied by the formation of germinal lymphoid centers and atrophy of glandular tissue with its replacement by adipose tissue.

Characterized by proliferation and metaplasia of the cells lining the ducts, with the development of so-called myoepithelial islands, which are found in biopsy specimens of the salivary glands in 30-40% of cases. The general lobular structure of these glands is usually preserved; in a number of patients some lobules are unchanged, others are almost completely destroyed. The size of the affected glands can be either enlarged or normal. It is important to note that, although clinical manifestations of Sjogren's syndrome are observed in a clear minority of patients with diffuse connective tissue diseases, subclinical histological signs of inflammation of the salivary glands are found in almost 100%. In particular, J. Waterhouse and J. Doniach (1966) found lymphocytic infiltrates in the submandibular glands in all those who died with rheumatoid arthritis.

Lymphoid infiltrates can in some cases occur not only in the exocrine glands, but also in the lungs, kidneys and skeletal muscles, sometimes leading to corresponding functional disorders. In some patients, lymphoid infiltrates in the salivary glands, lymph nodes and internal organs lose their usual inflammatory benign character.

The cells acquire a “younger” appearance and polymorphism, and show signs of invasive spread (in particular, the structure of the lymph nodes can be completely erased). In such cases, it is not always possible to clearly distinguish morphologically between benign and malignant lymphoproliferation, which is why the term “pseudolymphoma” arose. In some patients, the histological picture corresponds to immunoblastic lymphadenopathy; the development of true lymphosarcoma is also possible.

Clinical picture

A small amount of crumbly or thick viscous white or yellowish discharge is typical. In patients, tear production is reduced, up to the complete absence of tears, which is especially noticeable with severe negative emotions (inability to cry) and exposure to irritating substances (in particular, lack of tear production when peeling onions). As a rule, patients feel better in areas with a humid and cool climate.

Objective diagnosis of dry keratoconjunctivitis is possible by staining the altered areas of the conjunctiva and cornea with a 1% solution of rose bengal (the results of this technique, however, are not always interpreted unambiguously) and especially using a slit lamp, which makes it possible to reliably establish punctate and filamentous keratitis. To recognize decreased tear production, the most popular is the Schirmer test, which consists of the following.

A strip of filter paper 5 mm wide is placed with its upper end behind the lower eyelid at the border of its middle and inner third. Normally, paper gets wet by at least 15 mm in 5 minutes. With lower results, tear secretion can be stimulated by inhaling a 10% ammonium chloride solution, the bottle of which is placed 15 cm below the nose (“forced Schirmer test”). A positive Schirmer test (i.e. the very fact of insufficient tear production) is not synonymous with keratoconjunctivitis sicca, especially if this test is carried out at high temperatures in a dry room.

Clinically noticeable enlargement of the lacrimal glands in Sjogren's syndrome occurs in no more than 5% of patients. Dry eyes in this disease cannot be associated only with the destruction of the parenchyma of the lacrimal glands by the inflammatory infiltrate, since surgical removal of these glands (for other reasons) does not lead to such pronounced dryness. Apparently, the lesion of all mucous glands of the conjunctival sac in Sjögren's syndrome is of decisive importance in the development of xerophthalmia. Perhaps a change in the biochemical composition of tears may play a certain role: a decrease in viscosity and an increase in water content makes it impossible to create a uniform layer of tears over the surface of the cornea.

Untreated dry keratoconjunctivitis can be complicated by secondary infection, which is primarily due to the weakening or absence of the bactericidal effect of lysozyme contained in the normal secretion of the lacrimal glands. As a result of infection (bacterial or viral), multiple complications are possible - fusion of the eyelids with the eyeball, loss of the blink reflex, ulcerative keratitis, perforation of the cornea with the threat of uveitis, secondary glaucoma and loss of vision, which is very rare.

Xerostomia, if it exists in isolation, should not be considered as a mandatory manifestation of Sjögren's syndrome. Dry mouth occurs in older people, alcoholics, smokers, and people who breathe through their mouths or take antidepressants. Temporary severe dry mouth also occurs with nervous excitement. In addition, patients with Sjögren's syndrome may experience only very modest sensations of dry mouth and therefore do not actively complain about it. Moreover, even with indisputable histological signs of the disease, the amount of saliva sometimes remains sufficient for the patient not to experience discomfort.

Severe xerostomia in Sjögren's syndrome can be very distressing. The greatest difficulties arise when chewing and swallowing solid food, and therefore patients are forced to constantly drink water. Attempts to combat dryness by sucking lemon or sour candies are successful only in the early stages. In severe cases, food particles “stick” to the gums, cheeks and palate, and patients are forced to remove them with their hands. Dryness of the pharyngeal mucosa may cause dysphagia; in some cases, a true violation of the mobility of the esophagus was noted.

Painful cracks in the lips and corners of the mouth are very common. The mucous membrane of the mouth is often irritated, its surface tissue easily peels off, and eating hot or spicy food causes pain. When drinking milk, its coagulated threads that linger on the mucous membrane of the cheeks are sometimes incorrectly interpreted as fungal lesions (which in some cases can actually complicate xerostomia in Sjögren's syndrome). Painful atrophic glossitis with cracked tongue is not uncommon. Ulcerative stomatitis is also possible. Normal accumulation of saliva at the base of the frenulum of the tongue is not observed in clinically severe cases. It is also not possible to increase salivation after massage of the parotid and submandibular glands.

In most patients, enlargement of the parotid glands is unilateral and temporary, although it is possible to maintain normal size or to have bilateral enlargement. The submandibular glands are less frequently enlarged. Enlarged salivary glands are often slightly painful on palpation and are usually dense, with a smooth or uneven surface. At the same time, they do not have the stony density or nodularity characteristic of tumors. Acute pain in the glands, combined with fever, local hyperemia and hyperthermia, most often indicates the addition of a secondary infection, which is usually a consequence of the often observed dilation and tortuosity of the intraglandular salivary ducts.

To judge the pathology of these ducts, as well as the degree of atrophy of the gland parenchyma, contrast sialography can be successfully used. The severity of destruction of the glandular parenchyma is also determined by scintigraphy using labeled technetium (99m Te). In this case, the level of absorption of the radionuclide by the tissues of the salivary glands and, as a control, the thyroid gland is compared.

It is peculiar that the amount of saliva secreted per unit of time, which, it would seem, should characterize xerostomia especially accurately, is in fact not a sufficiently reliable indicator, since it is subject to sharp individual fluctuations. For a general judgment about a decrease in salivation, it is sometimes important to verify the small stimulating effect of acidic foods, such as lemon.

With an insufficient amount of saliva, gingivitis, bad breath, caries with toothache are common. Teeth crumble easily and previously placed fillings fall out. It should be borne in mind that drug-induced xerostomia is almost never the cause of dental pathology.

Changes in the mucous membranes of the external genitalia

Systemic manifestations of Sjogren's syndrome often present serious difficulties in their pathogenetic interpretation.

They can: 1) be symptoms of specific connective tissue diseases, with which Sjögren’s syndrome is often combined; 2) reflect the actual pathogenetic features of the syndrome in question and 3) be a nonspecific consequence of insufficient function of the exocrine glands with dry mucous membranes, etc. Obviously, the first category of symptoms does not relate to Sjögren’s syndrome as such and is not considered in this section.

Skin changes

Very often observed hives, which is a reflection of drug allergies characteristic of Sjögren's syndrome. K. Whaley et al. (1973) even found that allergic reactions to penicillin in RA almost exclusively occur in patients with concomitant Sjogren's syndrome.

Damage to joints and muscles

The most commonly affected joints are the knee and elbow joints. The pathogenesis of this variant of arthritis is unknown. There is an opinion about its immunocomplex nature. An indirect argument in favor of this point of view is the frequent combination of Sjogren's syndrome and RA, each of which is characterized by a high level of circulating immune complexes and the virtual absence of dry syndrome in patients with psoriatic arthritis and other seronegative spondyloarthritis, for which the formation of immune complexes is completely uncharacteristic.

It should be borne in mind that although in most patients with a combination of RA and Sjögren's syndrome, signs of the rheumatoid process long precede sicca syndrome, the opposite relationship is also possible. Therefore, at the first appearance of articular joints in a patient with long-term isolated Sjögren's syndrome, the onset of true rheumatoid arthritis cannot be ruled out.

Severe muscle damage in primary Sjögren's syndrome almost never occurs; there are only a few corresponding descriptions. Patients in some cases complain of mild myalgia and moderate muscle weakness, and the latter may also be associated with electrolyte disturbances and tubular acidosis. Muscle biopsy in patients with primary Sjogren's syndrome often reveals clinically asymptomatic focal myositis with perivascular lymphoid infiltration, as well as deposition of immunoglobulins and complement in muscle tissue and dystrophic changes in myofibrils under electron microscopy.

Respiratory system

Often the so-called dry bronchitis, in which thick mucous discharge causes a constant dry cough with difficulty in expectoration and the addition of a secondary infection. In the development of the latter, a certain importance is also attached to insufficient local IgA production.

Infiltrates of the walls of the bronchioles and alveolar septa by T and B lymphocytes are considered relatively specific for Sjögren's syndrome, which can clinically manifest as symptoms of airway obstruction with cough and shortness of breath, as well as episodes of usually mild interstitial pneumonia. Mild short-term pleurisy that resolves spontaneously within 1-2 weeks has often been described. Chronic interstitial inflammatory process (fibrosing alveolitis) is relatively rare.

The cardiovascular system in Sjögren's syndrome does not undergo any specific pathological changes primarily associated with this disease.

Kidney pathology

This view is supported by the fact that tubular acidosis in Sjögren's syndrome improves after treatment with cytostatic immunosuppressants. In addition, it is noteworthy that during an autoimmune crisis of rejection of a transplanted kidney, tubular acidosis also develops in combination with peritubular lymphoid infiltration. Since the involvement of the tubules in the process can manifest itself in a decrease in the response to antidiuretic hormone, when taking an increased amount of fluid (due to xerostomia), it sometimes leads to the development of a symptom complex of diabetes insipidus.

In rare cases, patients with Sjogren's syndrome develop acute glomerulonephritis, combined with hypocomplementemia and deposition of immunoglobulins and complement in the main membrane of the glomeruli. It is important to note that this is not about concomitant SLE, in which the development of Sjögren's syndrome is quite possible, since none of these patients had antibodies to native DNA, which are highly specific for patients with lupus nephritis.

Digestive and abdominal organs

A slight enlargement of the liver and spleen in combination with moderate laboratory signs of liver dysfunction is not so rare. Relatively often, antibodies to mitochondria, smooth muscle and membranes of liver cells are detected, which is considered a definite indication of the participation of the liver in the immune pathological process. The possibility of the development of a specific liver pathology (chronic active hepatitis or primary biliary cirrhosis) as a manifestation of Sjögren's syndrome (as some authors point out) does not seem to be proven sufficiently convincingly.

In such cases it cannot be ruled out reverse cause-and-effect relationships, since in these autoimmune diseases secondary Sjögren's syndrome often occurs (just as in RA in combination with dry syndrome, joint damage cannot be considered a manifestation of Sjögren's syndrome). Among the possible endocrine pathologies, one should point out histological signs of chronic thyroiditis of the Hashimoto type in approximately 5% of patients with Sjögren's syndrome. The autoimmune nature of both syndromes makes this combination quite understandable. At the same time, clinical signs of hypothyroidism are very rare.

A number of patients with severe Sjögren's syndrome experience vasculitis of medium and small vessels, manifested clinically by skin ulcers and peripheral neuropathy. It is characterized by a particularly frequent combination with the presence of Ro antibodies. In a number of patients, vasculitis is combined with hyperglobulinemia and is possibly a reflection of immune complex pathology (at least in some cases). Its main symptom is “hyperglobulinemic purpura,” manifested by foci of hemorrhages on the legs.

Elements of hemorrhages in typical cases, they are accompanied by itching and protrude above the surface of the skin, the temperature of which is usually slightly elevated. In rare cases, the rash appears confluent. Hyperglobulinemic purpura is very characteristic of the “dry syndrome”. It is believed that among patients with a combination of hyperglobulinemia and hemorrhages on the legs, 20-30% have or will develop typical manifestations of Sjogren's syndrome. Even with primary sicca syndrome, Raynaud's syndrome occurs more often than in the general population. However, its connection with vasculitis itself seems doubtful.

Changes in the central nervous system and cranial nerves are explained by most authors vasculitis corresponding localization. A wide range of cerebral symptoms has been described, including nystagmus, recurrent hemiparesis and, in particularly severe cases, serous meningoencephalitis. Among the cranial nerves, the trigeminal nerve is relatively more often affected with the development of a characteristic pain syndrome. In isolated patients with Sjogren's syndrome, severe necrotizing arteritis was observed with clinical manifestations corresponding to classic polyarteritis nodosa. However, it cannot be ruled out that polyarteritis in such cases was not a manifestation of Sjögren’s syndrome, but a “concomitant” disease (like RA, etc.).

Sigidin Ya.A., Guseva N.G., Ivanova M.M.

Today we’ll talk about the disease and Sjögren’s syndrome, you may have also heard the name “dry syndrome”. But some people still confuse the disease with Sjögren's syndrome.

Sjögren's disease is a systemic disease, the characteristic feature of which is chronic autoimmune damage to the salivary and lacrimal glands.

Sjögren's syndrome - a lesion of the salivary and lacrimal glands similar to Sjögren’s disease, developing in 5-25% of patients with systemic connective tissue diseases, more often in 50-75% of patients with chronic autoimmune liver lesions (chronic autoimmune hepatitis, primary biliary cirrhosis of the liver) and less often in others autoimmune diseases.

The disease is considered quite rare, with incidence ranging from 4 to 250 cases per 100,000 population. The peak incidence occurs between 35 and 50 years of age. Women get sick 8-10 times more often than men.

The diagnosis of Sjogren's disease is established on the basis of complaints, medical history, clinical, laboratory and instrumental examination of the patient, and always with the exclusion of other diseases, which will be discussed further.

Clinical features that increase the likelihood of Sjögren's disease .

Damage to secreting epithelial glands (autoimmune epitheliitis).

• Salivary glands: sialadenitis (usually mumps), submaxillitis or gradual enlargement of the parotid/submandibular salivary glands, rarely - minor salivary glands of the oral mucosa.
• Dry conjunctivitis/keratoconjunctivitis is observed in all patients, depending on the duration and stage of development of the disease.
• Damage to the red border of the lips - cheilitis, recurrent stomatitis, dry subatrophic/atrophic rhinopharyngolaryngitis.
• Damage to the epithelium of the biliary tract and renal tubular apparatus.

Extraglandular systemic manifestations

• Joint pain (arthralgia) is observed in 70% of patients. A third of patients experience recurrent non-erosive arthritis, mainly in the small joints of the hands.
• Vasculitis: ulcers, mainly on the legs, less often on the upper extremities and oral mucosa.
• Kidney damage: interstitial nephritis, glomerulonephritis, nephrotic syndrome.
• Damage to the peripheral nervous system: polyneuropathy, multiple mononeuropathy, mononeuritis, radiculoneuropathy, tunnel neuropathy.

Diagnostic methods.

To diagnose damage to the salivary glands, use:

• sialography of the parotid salivary gland with Omnipaque,
• biopsy of the minor salivary glands of the lower lip,
• biopsy of enlarged parotid/submandibular salivary glands,
• sialometry (measurement of stimulated saliva secretion),
• Ultrasound and MRI of the salivary glands.

To diagnose eye damage (keratoconjunctivitis sicca) use:

• Schirmer test (decreased tear production after stimulation with ammonia),
• staining the epithelium of the conjunctiva and cornea with fluorescein and lissamine green,
• determination of the stability of the tear film by the time of formation of “dry spots” on the cornea (normally more than 10 seconds).

Laboratory research:

• A decrease in the level of leukocytes (leukopenia) is a characteristic sign of the disease, often associated with high immunological activity of the disease.
• Increased ESR is detected in approximately half of patients.
• An increase in CRP is NOT characteristic of Sjögren's disease. High numbers are observed in severe cases of the disease and the development of complications.
• Rheumatoid (RF) and antinuclear factor (ANF) are detected in 95-100% of patients.
• Antibodies to Ro/SS-A and La/SS-B nuclear antigens are detected in 85-100% of patients using the enzyme immunoassay method. The simultaneous detection of Ro and La antibodies is most specific for Sjogren's disease, but is observed only in 40-50% of patients. La antibodies are more specific for the disease.
• Cryoglobulins are detected in a third of patients.
• A decrease in the C4 complement component is an unfavorable sign that affects the survival of patients with this disease.
• Increased IgG and IgA, less often IgM.

Criteria for the diagnosis of Sjögren's disease:

1. Eye damage - keratoconjunctivitis sicca
2. Damage to the salivary glands - parenchymal sialadenitis
3. Laboratory findings: positive rheumatoid factor or positive antinuclear factor (ANF) or presence of anti-SSA/Ro and/or anti-SSB/La antinuclear antibodies

Diagnosis certain Sjögren's disease can be diagnosed if the first two criteria (1 and 2) are present and at least one sign from criterion 3 is present, with the exception of autoimmune diseases (autoimmune hepatobiliary diseases).

Sjögren's syndrome can be diagnosed in the presence of a clearly verified autoimmune disease and one of the first two criteria.

Differential diagnosis

The symptoms of Sjögren's disease are not unique: dry eyes and dry mouth can occur with a variety of diseases and conditions.

Dry eyes: dry keratoconjunctivitis, hypovitaminosis A, chemical burns, blepharitis, damage to the fifth cranial nerve, wearing contact lenses, congenital and acquired diseases of the lacrimal glands, sarcoidosis, HIV infection, graft-versus-host disease, facial nerve paralysis, etc.

Dry mouth: medications (diuretics, antidepressants, antipsychotics, tranquilizers, sedatives, anticholinergics), anxiety, depression, sarcoidosis, tuberculosis, amyloidosis, diabetes mellitus, pancreatitis, lymphomas, dehydration, viral infections, radiation, postmenopause, etc.

Treatment.

Treatment goals: d achieving remission of the disease, improving the quality of life of patients, preventing the development of life-threatening manifestations of the disease.

Non-pharmacological approaches to dry syndrome:

1. Avoid situations that increase dryness of the mucous membranes: dry or conditioned air, cigarette smoke, strong wind, prolonged visual (especially computer), speech or psycho-emotional stress.
2. Limit the use of drugs that increase dryness and certain irritants (coffee, alcohol, nicotine).
3. Frequently drinking small amounts of water or sugar-free liquids can relieve dry mouth symptoms.
4. Stimulating saliva production using chewing gum and sugar-free candies is helpful.
5. Careful oral hygiene, use of toothpaste and rinses with fluoride, careful care of dentures, and regular visits to the dentist.
6. Therapeutic contact lenses provide additional protection to the corneal epithelium, but must be worn with adequate hydration.

Treatment of glandular manifestations of Sjogren's disease.

1. To replace the volume of tears, patients should use “artificial tears” 3-4 or more times a day. If necessary, the interval between instillation of tears can be reduced to 1 hour. To enhance the effect, it is possible to use artificial tears of higher viscosity, but such drugs are best used at night due to the effect of blurred vision.
2. Be sure to use antibacterial drops.
3. The use of saliva substitutes based on mucin and carboxymethylcellulose replenishes its lubricating and moisturizing functions, especially during night sleep (Oral balance gel, Biotene rinse, Salivart, Xialine).
4. Considering the high incidence of candidal infection in the presence of “dry syndrome”, local and systemic antifungal treatment is indicated.
5. Cyclosporine A ophthalmic emulsion (Restasis) is recommended for the treatment of keratoconjunctivitis sicca.
6. Local use of NSAIDs reduces discomfort in the eyes, however, it can provoke damage to the cornea.
7. Local use of glucocorticosteroids in short courses (up to two weeks) during exacerbation of keratoconjunctivitis sicca is considered acceptable.
8. Pilocarpine (Salagen) or cevimeline (Evoxac) are used to stimulate the secretion of the salivary and lacrimal glands.
9. Relief from dryness of the upper respiratory tract (rhinitis, sinusitis, laryngitis, bronchitis) is achieved by taking bromhexine or acetylcysteine ​​in therapeutic doses.
10. If pain occurs during sexual intercourse (dyspareunia), local use of lubricants is recommended, and in the postmenopausal period local and systemic use of estrogens is indicated.

Treatment of extraglandular systemic manifestations of Sjogren's disease.

To treat systemic manifestations of Sjogren's disease, glucocorticoids are used, alkylating cytostatic (leukeran, cyclophosphamide) and biological (rituximab) drugs.

1. Patients with minimal systemic manifestations are prescribed glucocorticoids in small doses or NSAIDs.
2. With a significant increase in the large salivary glands (after excluding lymphoma), the absence of signs of severe systemic manifestations, moderate and significant changes in laboratory activity indicators, it is necessary to prescribe small doses of glucocorticoids in combination with leukeran for several years.
3. In the treatment of vasculitis, cyclophosphamide is prescribed.
4. Severe systemic manifestations require higher doses of glucocorticoids and cytostatic agents in combination with intensive therapy.

Intensive therapy should be used for severe and life-threatening manifestations of the disease in order to stop high immunoinflammatory activity, change the nature of the course and improve the prognosis of the disease.

Use of genetically engineered biological drugs allows you to control systemic extraglandular manifestations of the disease and reduce functional glandular insufficiency.

Prevention.

Primary prevention is not possible due to the unclear etiology of the disease. Secondary prevention is aimed at preventing exacerbation, progression of the disease and timely detection of developing lymphomas. It provides for early diagnosis and timely initiation of adequate therapy. Some patients need to limit the load on the organs of vision, vocal cords and eliminate allergens. Vaccination, radiation therapy and nervous overload are contraindicated for patients. Electrical procedures should be used with great caution.

Sjögren's syndrome is one of the most common autoimmune disorders affecting the external secretion glands.

The salivary and lacrimal glands suffer, to a lesser extent, the glands of the mucous membranes.

With this disease, they cease to function fully and do not secrete secretions in the required quantity.

It is impossible to completely cure it, so therapy is limited to relieving symptoms. Sjögren's syndrome, also known as sicca syndrome, is usually associated with systemic immune-inflammatory diseases.

What it is

Who first diagnosed it?

The disease received its name in honor of the ophthalmologist who discovered and studied it, Heinrich Sjögren (whose last name is written and pronounced in Russian as Sjögren). Research began in 1929, when he came across a patient with a complex of characteristic symptoms.

Previously, they were not considered as signs of a systemic disease. The French ophthalmologist Henry Gougeroth helped the Swedish doctor study the syndrome.

Who is most susceptible to the disease?

Sjögren's syndrome is a fairly common disease around the world. As a result of observing patients suffering from it, scientists have not been able to find the reasons leading to its development, except that it may be a consequence of genetic predisposition.

The triggers for the onset of the disease can be stress, overwork and viral infections. Sjogren's syndrome is often found in combination with lupus erythematosus and rheumatoid arthritis.

Person's age and gender

Despite the fact that representatives of any race, age and gender are exposed to the disease, women over 40-50 years of age suffer the most. Men get sick much less often - they account for one in ten cases of Sjögren's syndrome, and children are very rarely affected.

Causes

Today, the exact cause of the development of Sjögren's syndrome is not known. But multiple observations and studies allow us to make the assumption that the disease appears due to a genetic predisposition. Antigens HLADW2, DW3, B8 are detected in patients.

Provoking factors for the development of “dry syndrome” include: viral infections, fatigue, hypothermia, neuropsychic stress.

Symptoms and diagnosis

The most common manifestations of Sjogren's syndrome are the following:

• xerostomia with normal or enlarged salivary glands;
• dry keratoconjunctivitis with normal or enlarged lacrimal glands;
• the presence of systemic connective tissue diseases.

Most doctors consider two of the three manifestations sufficient to make this diagnosis. The combination of “sicca syndrome” with a connective tissue disease is usually considered a secondary syndrome, and the presence of only xerostomia or keratoconjunctivitis sicca is considered a primary one.

Eye symptoms in Sjögren's syndrome occur as a result of decreased tear secretion. Patients complain of a burning sensation, a feeling of sand and scratching in the eyes. Some patients also experience redness of the eyelids and itching, decreased visual acuity, narrowing of the palpebral fissures, and accumulation of viscous secretions in the corners of the eyes.

With this disease, the cornea and conjunctiva of the eye become inflamed - dry keratoconjunctivitis develops. Also, with Sjögren's syndrome, enlargement of the salivary glands is often observed.

In some patients, due to the enlargement of the paired parotid glands, the oval of the face changes - in the literature this phenomenon is called the “hamster face”.

Typical symptoms of Sjogren's syndrome are dryness of the oral mucosa and lips, seizures, stomatitis, and multiple dental caries. While in the early stages of the disease, dry mucous membranes are observed only during physical exertion or anxiety, in the severe period this sensation is constantly observed. The mucous membranes become bright pink, are easily injured upon contact, and the tongue becomes dry.

Upon examination, a small amount of free saliva is revealed; it is viscous or foamy. Against this background, the appearance of a secondary infection (bacterial, viral, fungal) leads to the development of stomatitis.

The late stage of Sjogren's syndrome is characterized by severe dryness of the oral cavity, which leads to speech and swallowing disorders, cracked lips, folded tongue, keratinization of areas of the oral mucosa, and lack of free saliva.

Hypofunction of other endocrine glands, dryness of the nasopharynx, skin, vagina, development of bronchitis, tracheitis, esophagitis, atrophic gastritis and other diseases are also observed. In addition, articular syndromes such as polyarthritis or polyarthralgia, neuropathy of the facial nerve and trigeminal nerve, impaired sensitivity of the hands and feet, fever, hepato- and splenomegaly, hemorrhagic rashes on the trunk and limbs may be observed.

Treatment

Today there is no developed pathogenetic treatment for Sjögren's syndrome. For dry eyes, artificial tears are prescribed and do not require a prescription. The drug should be instilled four times a day or as needed.

Dry mouth is a very unpleasant symptom that causes severe thirst. Saliva is not only a moisturizing fluid that helps with chewing and swallowing food, it also protects the oral mucosa from infection. Due to the production of insufficient saliva, inflammatory processes of the gums and teeth, as well as caries, develop.

In this regard, it is necessary to ensure maximum oral care - brush your teeth several times a day, regularly rinse your mouth with special antiseptics, use sugar-free chewing gum.

In addition, timely dental prosthetics and caries treatment are important. It is also necessary to avoid taking medications that reduce salivary secretion - these include antidepressants, antihistamines and anticholinergics.

Sometimes pathogens can be transferred from the oral cavity to the salivary glands, resulting in their infection. This leads to swelling of the salivary glands, accompanied by pain and fever. If this condition develops, treatment with antibiotics, sprays and aerosols containing artificial saliva is prescribed.

To moisturize dry mucous membranes of the nasopharynx, the use of special aerosols is prescribed. In addition, during the treatment period it is necessary to maintain normal air humidity using a special humidifier or air conditioner.

Sometimes, if connective tissues are involved in the process (with damage to internal organs or the development of severe vasculitis), treatment of the disease consists of prescribing glucocorticoids or cyclophosphamide. For arthralgia, treatment with hydroxychloroquine is prescribed.

In case of gastric secretory insufficiency, long-term replacement therapy with natural gastric juice, hydrochloric acid, and pepsidil is prescribed. In case of pancreatic insufficiency, enzyme therapy is carried out: taking Panzinorm, Festal, etc.

Treatment with folk remedies

Sjögren's syndrome is a disease with a whole complex of manifestations and symptoms, so traditional methods of treatment can only be used to combat the symptoms.

For example, for dry eyes, different solutions with a composition close to tear fluid are used, and sea buckthorn and rose hip oil are used to heal the oral mucosa.

To ease the course of the disease, as well as to help with the main treatment, you can use traditional medicine recipes:

• To increase the amount of saliva secreted, it is worth adding to your diet foods that have a salivary effect - spices, mustard, lemon and any other fruits.
• Since Sjögren's syndrome is almost always accompanied by disturbances in the gastrointestinal tract, it is necessary to adhere to certain nutritional rules: treat foods more gently, include a sufficient amount of carbohydrates, proteins and vitamins in the diet, eat food 5-6 times a day in small portions.
• Dill or potato juice helps eliminate the effect of red eyes. To do this, moisten the bandage with freshly prepared juice and apply it to your eyes for 20 minutes. After the procedure, you should not strain your eyes for several hours. It is recommended to wear dark glasses, which will help reduce discomfort from too bright light.

Before using traditional recipes to treat any disease, you must consult with herbalists and your doctor to avoid negative consequences.

Prevention

Patients are subject to clinical observation by a rheumatologist, as well as observation by a dentist, ophthalmologist and gynecologist. Preventive treatment with symptomatic drugs and medication to achieve a state of remission are necessary.

Primary prevention of the disease has not been developed to date.

Prognosis and complications

The course of the disease is not life-threatening, but significantly worsens the quality of life. Therapy prescribed in a timely manner can slow down the progression of pathological processes and preserve the ability of patients to work. In cases of lack of treatment, complications often occur that lead to disability.

It should be noted that primary lesions with Sjögren’s symptom are often accompanied by secondary infections, which result in the development of bronchopneumonia, recurrent tracheitis, and sinusitis. With systemic lesions, the development of circulatory disorders of the spinal cord and/or brain and renal failure is likely.

Conclusion

Despite the fact that the disease is quite serious and causes great discomfort, it is not fatal, and if you consult a specialist in time and start treatment, you can live with it, practically not noticing the presence of a problem.

It is worth remembering about a healthy lifestyle, this will not only improve immunity and prevent the pathological process from progressing quickly, but will also significantly reduce the risks of other concomitant pathologies.

Video

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Bibliography

1. Ophthalmology. Clinical recommendations Ed. V.V. Neroeva 2019
2. Ophthalmology. National leadership. Brief edition Ed. S.E. Avetisova, E.A. Egorova, L.K. Moshetova, V.V. Neroeva, Kh.P. Takhchidi 2019

Sjogren's syndrome (“dry syndrome”) is manifested by a decrease in the function of the exocrine glands; as a result of this pathology, severe dryness of the skin and mucous membrane of the vagina, trachea, nasopharynx, eyes, and oral cavity appears, and a decrease in the secretion of digestive enzymes produced by the pancreas is also observed.

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

According to recent epidemiological studies, the presence of this disease can be detected in 0.59-0.77% of the total population of the planet, while among patients over the age of 50 years, the pathology occurs in 2.7% of cases. The incidence of females exceeds the incidence of males by 10-25 times.

Causes and mechanism of development of Sjogren's syndrome

The most characteristic of Sjögren's syndrome is damage to the lacrimal and salivary glands and a decrease in their function. As a result, inflammation and dryness of the mucous membrane of the conjunctiva and oral cavity (xerostomia), keratoconjunctivitis, keratitis (inflammation of the cornea of ​​the eyes) occurs.

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

The viruses themselves and the epithelial cells of the glands changed by their influence are perceived by the immune system as antigens (foreign agents). The immune system produces antibodies against such cells and gradually causes the destruction of gland tissue. The disease often occurs as a hereditary or familial disorder, particularly among twins, suggesting a genetic predisposition.

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

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 of age, while girls are most often affected among children;

immune control using T lymphocytes;

viral;

genetic.

Symptoms of Sjögren's syndrome

All symptoms of Sjögren's syndrome are divided into two groups:

systemic, or extraglandular - various symptoms that indicate damage to various body systems and are not characteristic of this pathology;

glandular - damage to the epithelial cells of the secreting glands, which is accompanied by a decrease in the functions of the latter.

A mandatory and constant symptom for diagnosing Sjögren's syndrome is damage to the salivary and lacrimal glands. In the early stages of the disease, dry mucous membranes are felt by the patient mainly during anxiety or increased physical activity; as the pathology progresses, the feeling of dryness becomes permanent, forcing the patient to drink food, moisten the mouth very often and use drops like artificial tears to moisten the eyes.

Glandular symptoms

Among the manifestations of keroconjunctivitis are redness and itching in the eyelid area, a burning sensation, sand and scratching in the eyes, and periodic accumulation of white secretion in the corners of the eyes. A decrease in visual acuity develops, pinpoint swelling (infiltrates) and hemorrhages of the conjunctiva, narrowing of the palpebral fissure, increased sensitivity to bright light - pain and pain in the eyes, lacrimation, discomfort.

Due to deep dryness of the cornea, clouding occurs, and corneal trophic ulcers begin to form due to impaired nutrition of the eyes. A staphylococcal infection occurs and purulent conjunctivitis develops; suppuration of the ulcers leads to a rather serious complication in the form of perforation. Rarely, the lacrimal glands themselves become enlarged.

Chronic parenchymal parotitis

It is an obligatory and second constant sign of Sjögren's syndrome and is characterized by widespread damage to the tissues of the salivary glands. Quite often, the precursor to mumps is an enlargement of the cervical and submandibular lymph nodes, caries of many teeth, dryness and redness in the corners of the lips, and stomatitis.

Then there is dryness of the oral mucosa and an increase in the size of the parotid salivary glands, less often - the sublingual, submandibular, palatine glands. Dryness in the mouth can develop initially with minor physical or psycho-emotional stress, but with the progression of the pathology it becomes permanent and requires constant moistening of the mouth when eating, talking, especially if sweet foods are consumed.

The disease in half of the patients progresses with the development of exacerbations, during which the parotid salivary glands enlarge and provoke changes in the contours of the face (the so-called “hamster face”). The glands may be slightly painful on palpation or completely painless. During the period of remission, the glands decrease in size, but during the next exacerbation they can remain enlarged all the time. Approximately 30% of patients experience enlargement of the glands not only with relapse of the pathology, but constantly and gradually.

The mucous membrane of the mouth and tongue becomes red and dry (lacquered), sometimes bleeds and is easily injured, while the saliva that is released is scanty, viscous and foamy.

In case of no treatment:

the listed symptoms become more pronounced;

the papillae of the tongue atrophy and smooth out, folds appear on the tongue, glossitis develops (inflammation of the root of the tongue), which makes swallowing significantly more difficult;

areas with keratinized epithelium appear on the mucous membrane of the cheeks, saliva is completely absent;

a viral, fungal or bacterial infection may occur;

crusts and cracks form on the lips;

teeth fall out completely or partially.

Diagnosis of Sjöngren's syndrome is based on the listed group of symptoms; other signs may accompany the pathology in various variations, but are not decisive in making the final diagnosis.

Associated signs of glandular damage

Dryness of the mucous membrane of the upper respiratory tract.

Causes hoarseness, provokes the development of chronic rhinitis and inflammation of the mucous membrane of the paranasal sinuses, and hearing loss due to otitis media. Swelling, redness, dryness and atrophy of the vaginal mucosa leads to the development of chronic colpitis, which is accompanied by a decrease in libido, itching, pain, and burning.

A common symptom is a decrease in sweating, as well as dry skin.

About 30% of patients with this syndrome suffer from damage to the apocrine sweat glands, which are located in the area of ​​the external genitalia, pubis, lower parts of the anterior abdominal wall, and armpits. This leads to pigmentation and peeling of the skin in these areas, phlegmons and abscesses are formed in the event of a secondary infection.

Symptoms of pathologies of the digestive organs (develops in 80% of cases).

This is a number of diseases, including chronic cholecystpancreatitis, gastritis, chronic esophagitis. These pathologies manifest themselves as pain in the right hypochondrium and epigastric region, pain behind the sternum during the passage of food, belching, nausea, which in some cases ends in vomiting. If there is a decrease in the secretion of digestive enzymes by the pancreas, intolerance to dairy and fatty products appears, and a clinical picture of impaired intestinal function also develops.

Extraglandular symptoms

This category includes pain in the bones in the absence of changes in them (determined using x-rays). In 60% of patients, there is pain, stiffness and limitation of movement in small joints, which is especially noticeable in the morning; the mobility of large joints is less often affected. 5-10% develop pain and mild muscle weakness, and sometimes severe widespread inflammation of muscle groups (polymyositis) may occur.

In 50% of cases of Sjöngren's syndrome, tracheobronchitis develops, which is accompanied by shortness of breath and coughing; radiographs in 65% of patients reveal pulmonary fibrosis, chronic interstitial pneumonia, and less often - dry and effusion (exudative) pleurisy.

Most patients are diagnosed with enlarged supraclavicular, cervical, submandibular, occipital lymph nodes; in 30-35%, enlarged lymph nodes (lymphadenopathy) are generalized. In such cases, there is an enlargement of the liver and spleen.

The same number of patients suffer from vasculitis (inflammation of the inner lining of the arteries), which occurs under the guise of erased forms of Raynaud's syndrome or atherosclerosis (obliterating) of the vessels of the lower extremities. Damage to small vessels manifests itself in the form of a variety of spotty or pinpoint skin rashes, which are accompanied by itching, fever, burning, the formation of ulcers and dead skin areas.

Polyneuropathy (a common lesion of the peripheral nervous system) is clinically manifested by a disorder or complete loss of sensitivity of the skin in the area of ​​​​the feet and hands (like “socks” and “gloves”), less often neuritis of the trigeminal nerve, facial nerve, damage to the vessels of the membranes of the spinal cord and brain can develop .

Also, thanks to the examination, it is possible to detect signs of damage to the thyroid gland and a decrease in its function by up to 10%, and the emergence of a tendency to allergic responses to many household chemicals, medications, and foods.

Diagnostics

The diagnosis of “Schöngren’s disease” is made based on the presence of two main diagnostic indicators - parenchymal parotitis and ceraconjunctivitis, but only after the third criterion has been excluded - a systemic autoimmune disease (rheumatoid polyarthritis, systemic lupus erythematosus). If the third indicator is present, the diagnosis sounds like “Schöngren’s syndrome.”

Laboratory research methods are considered additional. They make it possible to perform differential diagnostics and, to some extent, determine the degree of activity of the pathology:

the presence of antibodies to components of cell nuclei, which are determined using the immunofluorescence method;

presence of rheumatoid factor;

increased levels of immunoglobulins M, G, A in the blood;

decrease in blood platelets and leukocytes, anemia;

high erythrocyte sedimentation rate (ESR).

The Schirmer test is also used, which allows you to determine the degree of reduction in tear secretion after stimulation of lacrimation with ammonia, staining the conjunctiva and cornea with special ophthalmic dyes, followed by biomicroscopic examination of epithelial cells.

To study the functionality of the salivary glands, use:

microscopic examination of biopsy material (biopath) of the salivary gland;

sialography - carried out using an x-ray method after introducing a contrast agent into the ducts of the salivary glands, the technique allows you to identify cavities in the gland whose diameter exceeds 1 mm;

methods of stimulated and unstimulated sialometry - the amount of saliva that is secreted in a certain unit of time.

Treatment of Shengren's syndrome

Treatment of this syndrome is carried out based on the stage of the pathology and the presence of systemic manifestations.

In order to stimulate the functions of the gland, the following is carried out:

subcutaneous administration of galantamine;

drip administration of contrical;

as symptomatic therapy, an “artificial tear” drug (eye drops) is prescribed - with high (Oftagel, Vidisik, Lakropos), medium (Lakrisin), low viscosity (Natural Tear, Lacrisif) ;

For general strengthening of the body, a course of vitamin therapy is carried out.

In the initial stages, in the absence of damage to other body systems and unexpressed changes in laboratory tests, long courses of glucocorticosteroids (Dexamethasone, Prednisolone) in small doses are prescribed.

If laboratory indicators and symptoms are significantly expressed, but there are no systemic manifestations, cytostatic immunosuppressive drugs - Azathioprine, Chlorbutin, Cyclophosphamide - are added to corticosteroid therapy. Maintenance therapy is carried out with the same drugs for several years.

If there are symptoms of systemic damage to the body, depending on the stage of the disease, high doses of immunosuppressants and corticosteroids are immediately prescribed for several days with a gradual transfer of the patient to maintenance doses.

In case of kidney damage, vasculitis, generalized polyneuritis and other severe manifestations of pathology, extracorporeal treatment is added to the above treatment - plasma ultrafiltration, hemosorption, plasmapheresis.

Other drugs are prescribed depending on the presence of complications and concomitant pathologies - endocervicitis, pneumonia, gastritis, cholecystitis. In some cases, dietary restrictions and limited physical activity are required.

Sjögren's syndrome is not a life-threatening condition, but it can reduce the quality of life so much that a person becomes disabled. Timely diagnosis and therapy at an early stage of this condition can significantly slow down the development of the pathological process and prevent severe complications, preserving the person’s ability to work.

Most disruptions to the functioning of a living organism are provoked by specific external stimuli, but there is a separate category of diseases when our immunity begins to destroy normal cells on a large scale. This indicates a failure directly in the process of recognizing native and foreign cells. Defense forces work against connective tissue cells, causing malfunction of many organs - this is Sjögren's syndrome.

What is Sjögren's syndrome

Sjögren's disease or syndrome is an autoimmune disease that affects connective tissue in various body systems. The syndrome belongs to the group of collagenoses. These are the same type of functional and morphological changes in tissue, mainly in its collagen fibers. Collagenosis is characterized by constant progression and involvement of organs, structures of the musculoskeletal system, skin and blood vessels. Sjogren's disease is recognized as the most common among all collagenoses.

Most often, Sjogren's disease is detected in women 20-60 years old, although mainly after 40, when hormonal levels change due to menopause. The specific causes of autoimmune failure have not been clarified, but doctors identify hereditary predisposition and an aggressive response to infection as the most likely.

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There is a theory that the destruction of native cells by the immune system is caused by various viruses. They act asymptomatically, integrating their proteins into the cell membranes of healthy cells, causing the defense system to perceive them as foreign and attack. This mechanism is the basis of many diseases, but systemic connective tissue disorders have the most autoimmune factors.

Connective tissue acts as the “foundation” for all organs; many metabolic and immune processes are ensured through it. Therefore, connective tissue diseases are classified as very severe diseases that greatly affect a person’s life. If arthritis is localized in the joints, rheumatism in the heart, and ankylosing spondylitis in the spine, then with Sjogren's syndrome the glands of the lymphatic system and mucous membranes are affected.

The disease begins with an aggressive immune response, when antibodies to normal cells appear and infiltration of the glandular ducts begins. The exocrine glands (salivary, lacrimal, vaginal) are mainly affected. When the disease spreads to the entire body, a third of patients are diagnosed with myositis (inflammatory damage to muscle tissue), nephritis (inflammatory destruction of the kidneys), vasculitis (productive or productive-destructive), and pneumonia. Sjögren's disease may be detected by screening for arthritis, Hashimoto's thyroiditis, and systemic lupus.

Forms and stages of Sjögren's syndrome

There are subacute and chronic forms of the disease. For subacute, extraglandular symptoms are more characteristic: rheumatological disorders, vasculitis and corresponding skin lesions, malfunctions of the kidneys and PNS. Chronic is characterized by a predominance of glandular abnormalities: improper functioning of the glands, diseases of the ENT organs.

According to the severity of symptoms, early, severe and late stages are distinguished. Negative changes in connective tissue occur with varying intensity. With a high degree of intensity, symptoms of mumps, stomatitis, conjunctivitis and arthritis are noted. In severe cases, generalized lymphadenopathy (enlarged nodes of the lymphatic system) and hepatosplenomegaly (enlarged spleen and liver) are diagnosed. Based on the results of laboratory tests, a violent inflammatory process is revealed.

Moderate intensity is expressed in a decrease in the immune response, there is extensive destruction of the epithelium of the glands. Low intensity causes xerostomia (saliva deficiency), (inflammatory damage to the conjunctiva and cornea) and gastritis (inflammation of the gastric mucosa).

Symptoms of Sjögren's disease

At first, a person feels only dry eyes and occasional blurred vision. Over time, these symptoms are accompanied by dry mouth, and the need to drink food appears. The formation of small ulcers in the oral cavity and multiple caries is possible. Without treatment, the symptoms worsen, affecting the genitals, digestive and respiratory systems. Symptoms of gastritis, colitis, bronchitis, as well as muscle discomfort appear. In the advanced stage, nerve damage and Raynaud's syndrome are possible, when a person suffers from chilliness, a feeling of wet limbs and goosebumps.

Symptoms of Sjögren's disease:

1. Ophthalmological symptoms. Insufficiency of the lymphatic system is expressed in a decrease in the production of tear fluid. Drying of the mucous membrane is accompanied by burning and itching. There may be redness of the eyelids, accumulation of discharge, and blurred vision. With a long course of the disease, conjunctivitis or keratoconjunctivitis sicca develops. A complication may be perforation of the cornea in the presence of an ulcer. Gradually, dystrophy of the cornea and conjunctiva begins, filamentous keratitis or xerosis (drying of the eye mucosa) develops.
2. Symptoms of ENT organs. Characteristic signs of Sjögren's syndrome: pronounced coloring of the oral mucosa, dry tongue, seizures, insufficiency of free saliva, stomatitis, extensive dental damage. At first, discomfort is felt only after physical exertion, but later it persists constantly. Swallowing disorders occur, the tongue becomes covered with folds, and foci of keratinization are visible on the mucous membrane. Secondary infection leads to the development of severe viral, fungal or bacterial stomatitis. Cheilitis and dry nasopharyngolaryngitis are also possible. Involvement of the respiratory system is accompanied by a change in voice and the appearance of hoarseness.
3. Lymphatic symptoms. Insufficient functionality of the endocrine glands causes dryness of the skin, nasal passages, pharynx, vulva and vagina. Tracheitis, bronchitis, esophagitis and gastritis may develop.
4. Neurological symptoms. If connective tissue is damaged, neuropathy of the trigeminal and facial nerves is possible. The development of generalized vasculitis is reflected in the functioning of the peripheral nervous system, which is expressed in polyneuropathy, mononeuropathy, mononeuritis, radiculoneuropathy.
5. Rheumatological symptoms. Articular syndrome occurs as polyarthralgia or polyarthritis. There are regular recurrences of swelling and pain in the joints. 70% of patients with Sjogren's syndrome have arthralgia, and 30% have non-erosive arthritis of the small joints of the hands of a recurrent nature. Possible muscle damage (myositis) and impaired sensitivity of the limbs.
6. Skin symptoms. Hemorrhagic rash, increased dryness of the skin, cracks (with a decrease in air temperature). Purpura is observed in 30% of patients (hypergammaglobulinemic in lymphocytic vasculitis and cryoglobulinemic in destructive). Against the background of destructive vasculitis, ulcerations on the legs also occur.
7. Other symptoms. Manifestations of the syndrome include hepatomegaly (enlargement and hardening of the liver) and splenomegaly (enlargement of the spleen). Many are diagnosed with interstitial nephritis or glomerulonephritis with nephrotic syndrome.

Damage to the salivary glands in this connective tissue disease occurs as sialadenitis or mumps, less often the symptoms resemble submaxillitis or sialodochitis. Sometimes there is an increase in small glands, much more often the parotid or submandibular glands are affected, which noticeably changes the oval of the face.

Diagnosis of Sjögren's syndrome

The examination begins with examining the patient. The specialist should identify dry skin and tongue, the presence of ulcers on the oral mucosa and multiple caries, inflammation of the eyes and decreased tear production. Systemic damage to the connective tissue causes swelling of the parotid glands. Periodic swelling of the joints is possible, and upon palpation - pain. It is recommended to additionally listen to the lungs for wheezing.

When diagnosing Sjogren's syndrome, you need to consult a therapist, rheumatologist, ophthalmologist, dentist and other specialists. The dentist can clarify the nature of the damage to the salivary glands, excluding diseases with similar symptoms. An ophthalmologist conducts a test to assess the functionality of the lacrimal glands: exposure to ammonia reduces the production of tear fluid. Marking of ocular structures makes it possible to identify erosion and dystrophy.

Laboratory tests to detect Sjögren's syndrome:

1. General blood analysis. This syndrome is characterized by severe leukopenia, that is, a reduction in the number of leukocytes. Leukopenia indicates increased immune activity and the presence of anti-leukocyte antibodies. Almost all patients have mild or moderate anemia, and half have an increase in ESR. This is due to dysproteinemic disorders. An increase in CRP is not typical for Sjogren's syndrome (excluding effusion serositis, glomerulonephritis, destructive vasculitis, demyelinating neuropathy, intercurrent infection).
2. General urine analysis. When Sjögren's disease is combined with urolithiasis or chronic pyelonephritis, the addition of a secondary infection causes leukocyturia (the presence of leukocytes in the urine). Characteristic signs of kidney damage include the presence of protein, glucose, red blood cells, casts in the urine, as well as a decrease in specific gravity and increased acidity of urine.
3. Blood chemistry. There is a high concentration of γ-globulins, an excess of proteins, fibrin, sialic acids and seromucoid. Increased aminotransferase activity and cholestasis at an early stage of disease development may indicate concomitant viral or autoimmune hepatitis, cholangitis, liver or pancreas pathologies. If the total protein concentration increases and an M gradient is detected, additional diagnostics for plasma dyscrasias are necessary.
4. Immunological reactions. Rheumatoid factor is detected in all patients, and its high titers indicate the presence of cryoglobulinemic vasculitis and the formation of MALT tissue. Cryoglobulins are detected in 1/3 of patients. Sjögren's syndrome is characterized by the following picture: an excess of IgG/IgA and IgM antibodies, the presence of antibodies to DNA, collagen and epithelium, the presence of lupus cells, an excess of B-lymphocytes and a lack of T-lymphocytes. Almost all have autoantibodies to nuclear antigens (Ro/SS-A and La/SS-B).

Unfavorable signs in Sjögren's syndrome include a decrease in complement C4, which signals the active development of cryoglobulinemic vasculitis. A sharp increase in the concentration of IgM immunoglobulins may indicate the concomitant development of lymphoma. If a patient has autoantibodies to thyroglobulin and thyroid peroxidase, it is necessary to diagnose autoimmune thyroiditis. In case of chronic liver dysfunction, tests reveal antimitochondrial antibodies, as well as antibodies to smooth muscles and microsomes (liver and kidney tissue).

Additional examination methods:

• sialography or contrast radiography;
• biopsy and ultrasound of the salivary glands;
• MRI of glands;
• X-rays of light;
• gastroscopy (checking the functioning of the esophagus and stomach);
• echocardiography (checking the functioning of the heart).

Ultrasound examination of the salivary glands makes it possible to assess their volume and structure, as well as identify inflammation, cysts and stones in the ducts. Magnetic resonance imaging provides the greatest amount of information about the functioning of the salivary glands. Chest X-rays are often uninformative. Computed tomography of the chest is effective only at an early stage of the disease, when the respiratory system is involved in the inflammatory process. Ultrasound examination of the abdominal organs can identify the causes of dry mouth and throat.

Patients with Sjögren's syndrome should visit a rheumatologist, ophthalmologist, and dentist regularly. It is advisable to see a gastroenterologist, since 80% of people with this disease also suffer from gastrointestinal disorders. At risk are people with a long history of Sjogren's disease and concomitant diffuse atrophic gastritis.

Basic and symptomatic treatment of Sjögren's syndrome

A rheumatologist examines patients and prescribes therapy for Sjogren's syndrome. The process may require consultation with a nephrologist, ophthalmologist, gynecologist, gastroenterologist, pulmonologist and other specialists. Therapy is based on hormones and cytostatic agents with immunosuppressive effects. A combination of Prednisolone with Chlorobutin or Cyclophosphamide is effective.

Symptomatic treatment for Sjogren's disease consists of preventing secondary infection and eliminating excessive dryness. Most people with this disease manage to live normally, only occasionally it manifests itself more severely and can cause death. When the systemic manifestation of the syndrome is pronounced, glucocorticoids, immunosuppressants and other drugs are added to the prescription.

Symptomatic treatment for Sjogren's disease:

1. Extracorporeal hemocorrection for the treatment of concomitant vasculitis, cerebrovasculitis, polyneuritis or glomerulonephritis. Depending on the circumstances, plasmapheresis, hemosorption or plasma filtration are performed.
2. Artificial saliva and oil solutions to moisturize and restore the oral mucosa. Blockades with novocaine, calcium preparations and ointments (Methyluracil, Solcoseryl) are effective.
3. Artificial tears and regular eye rinsing with antiseptic agents. It is possible to use soft contact lenses to prevent dystrophy.
4. Medicinal applications with antimycotics (antifungal drugs) or antibiotics against inflammation in the parotid glands.
5. Enzyme therapy for malfunctioning pancreas. Adequate prevention of diabetes mellitus.
6. The use of hydrochloric acid and gastric juice for gastric insufficiency. Pepsin is also used.
7. Diet and additional consumption of yoghurts with lactobacilli to prevent stomatitis, vaginitis and similar complications.
8. Daily use of fluoride gels to protect dental tissue.

To moisturize the eyes, you can use medications (drops and gels) sold in pharmacies without a prescription. Eye comfort is also ensured by increasing humidity in home and work areas. The patient is not recommended to sit near heaters and air conditioners. On the street you need to wear glasses and protect your eyes from solar radiation in every possible way.

To moisturize the oral cavity, you need to drink a lot of clean water or spray a glycerin solution into your mouth (20 ml of product per 1 liter of water). You can stimulate saliva production with water and lemon juice, lollipops, or regular chewing gum. It is important that these products do not contain sugar or harmful ingredients. Warm saline solutions help to painlessly moisturize and cleanse the nasal passages.

To eliminate vaginal dryness, there are various hypoallergenic suppositories, ointments and gels. It is better to ask your gynecologist for a prescription for the drug during a routine examination, so as not to worsen the situation. If the skin is hypersensitive, it is not recommended to take a hot shower or bath and wipe dry with a rough towel. After water treatments, you can apply a moisturizer or special oil to the skin to retain moisture.

Prognosis and possible complications

Sjögren's syndrome does not pose a serious threat if treated correctly, but its manifestations can complicate a person's life. It is necessary to eliminate symptoms in a timely manner and carry out supportive procedures so that a person does not become disabled due to severe complications. Only through complex treatment can it be possible to slow down the destruction of connective tissue and maintain a person’s ability to work.

Complications and consequences of Sjogren's syndrome:

• secondary infection;
• mumps;
• sinusitis;
• blepharitis;
• bronchitis;
• bronchopneumonia;
• tracheitis;
• diabetes;
• kidney failure;
• thyroid dysfunction;
• circulatory disorders in the brain.

Prevention of Sjögren's disease

There is no specific prevention against connective tissue diseases, since medicine has not identified the exact causes of the development of the disease. Doctors have proposed a list of general measures that will help reduce the risk of developing the disease or detect it at an early stage. The main role in this list is given to the prevention of infection, especially rotavirus infections. Women over 40 and people with a family history of Sjögren's syndrome need to take prevention seriously.

Basic preventive measures:

• regular visits to specialists, contacting doctors for symptoms;
• prevention and treatment of infectious diseases, especially ENT infections;
• treatment and control of all associated disorders;
• taking all medications as prescribed by the doctor;
• increasing stress resistance, avoiding emotional overload;
• limiting stress on the visual system, speech and digestive organs;
• timely treatment to the hospital in case of exacerbation;
• refusal of any type of tanning and radiation therapy;
• physiotherapeutic procedures and vaccinations only after the approval of the attending physicians.

Sjögren's syndrome is common and dangerous. The disease is accompanied by many unpleasant symptoms and requires constant treatment with drugs that suppress the immune system. Only the joint efforts of a rheumatologist, dentist, ophthalmologist and gastroenterologist can maintain a stable condition and performance in Sjögren’s disease.

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