Sjögren's syndrome is an autoimmune disease that causes systemic damage to connective tissues. Exocrine glands - salivary and lacrimal - suffer most from the pathological process. In most cases, the disease has a chronic progressive course.

Sjögren's syndrome - what kind of disease is it?

The Swedish ophthalmologist Sjogren first drew attention to the complex of symptoms of dry syndrome 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. Observation interested other doctors and scientists. It turned out that this pathology is common and specific treatment is required to combat it.

Sjögren's syndrome - what is it? This chronic autoimmune disease appears against the background of malfunctions of the immune system. The body takes some of its own cells as 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 external secretion glands - usually salivary and lacrimal.

Sjögren's syndrome - causes

To say unequivocally why autoimmune diseases develop, medicine cannot yet. Therefore, where the dry Sjögren'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, the impetus for the development of the disease is viruses - cytomegalovirus, Epstein-Barr, herpes - or diseases such as polymyositis, systemic scleroderma, lupus erythematosus, rheumatoid arthritis.

Primary Sjögren's syndrome

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

Secondary Sjögren'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 connective tissue lesions.

Sjögren's syndrome - symptoms

All manifestations of the disease are usually divided into glandular and extra-glandular. The fact that Sjögren's syndrome has spread to the lacrimal glands can be understood by the sensation of burning, "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 occurs, the palpebral fissures noticeably narrow, visual acuity deteriorates. Enlargement of the lacrimal glands is a rare phenomenon.

Characteristic signs of Sjögren's syndrome, which affected the salivary glands: dryness of the mucous membrane in the mouth, red border, lips. Often, patients form, and, in addition to salivary glands, some nearby glands also increase. At first, the disease manifests itself only during physical exertion or emotional overstrain. But later, dryness becomes permanent, the lips become covered with crusts that crack, which increases the risk of a fungal infection.

Sometimes, due to dryness in the nasopharynx, crusts begin 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 become 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.

Extra-glandular manifestations of the symptom complex of Sjögren's syndrome look like this:

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

Sjögren's syndrome - differential diagnosis

The definition of the disease is mainly based on the presence of xerophthalmia or xerostomia. The latter is diagnosed using sialography, parotid scintigraphy and salivary gland biopsy. For the diagnosis of xerophthalmia, the Schirmer test is performed. One end of a strip of filter paper is placed under the lower eyelid and left 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, percynos anemia, drug-induced disease. The identification of primary dry syndrome is significantly facilitated by the detection of SS-B antibodies. It is most difficult to diagnose Sjögren's disease with rheumatoid arthritis, because joint damage begins long before signs of dryness appear.

Sjögren's syndrome - tests

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

1. In the general blood test, an 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 revealed 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. The results of a salivary gland biopsy confirm the symptoms of Sjögren's syndrome.

Sjögren's syndrome - treatment

This is a serious problem, but it is not fatal. If you pay attention to its signs in time and start treatment with a diagnosis of Sjögren'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 strengthen the immune system, will not allow the pathological process to actively develop and significantly reduce the risk of complications.

Can Sjögren's syndrome be cured?

Once the diagnosis is confirmed, the patient receives therapeutic advice. Sjögren's syndrome is 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 appointments help, laboratory parameters and histological picture improve.

Dry syndrome therapy involves alleviating symptoms and, if necessary, combating an underlying autoimmune disease. Before treating Sjögren's syndrome, a diagnosis is necessarily carried out. After that, as a rule, such means are used:

• glucocorticoid (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;
• Kontrykal or Trasilol - drugs that stop the production of proteolytic enzymes;
• Cyostatics - Chlorbutin, Azathioprine, Cyclophosphamide (mostly recommended to be taken together with glucocorticoids).

To get rid of dry mouth, rinsing is prescribed. Dry eye syndrome is treated by instillation of saline solution, Hemodez. Overdried bronchi and trachea can help the drug. Applications with Dimexide, Hydrocortisone or Heparin fight inflammation of the glands. Sometimes dry mouth with a diagnosis of Sjögren's syndrome leads to the development of dental diseases. To prevent them, you need to take care of maximum oral hygiene.

Sjögren's disease - treatment with folk remedies

Dry syndrome is a complex of symptoms and signs. With all of them it is better to fight traditionally. But sometimes, with Sjogren's syndrome, alternative methods used in parallel help to improve the patient's condition. Some patients, for example, note that dill and potato juice eye drops are much more effective than pharmacy lacrimal fluids.

Sjögren's syndrome

What is Sjögren's Syndrome -

Sjögren's syndrome ("dry 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. Among clinicians, the diagnostic triad of K. Bloch et al. is popular:

• dry keratoconjunctivitis with enlarged or normal lacrimal glands;
• xerostomia with enlarged or normal salivary glands;
• the presence of a systemic connective tissue disease (more often RA, less often SLE, SJS, 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 "dry syndrome" with RA or other connective tissue disease is considered as a secondary Sjogren's syndrome, and the presence of only dry keratoconjunctivitis and xerostomia as a primary Sjogren's syndrome (or "Sjogren'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.

Pathogenesis (what happens?) during Sjögren's Syndrome:

The pathogenesis has not been elucidated. It is known that T and B lymphocytes are present in the foci of tissue lesions; local synthesis of a large number of immunoglobulins is characteristic, which suggests a decrease in the function of T-suppressors and, accordingly, activation of B-cells. Among patients with primary Sjögren's syndrome, there is a higher frequency of DR3 and B8 histocompatibility antigens. Indirect evidence of the role of heredity and autoimmune disorders in Sjögren's syndrome are frequent manifestations of dry syndrome in purebred NZB/W and MRL mice with clear signs of autoimmune pathology.

The main histomorphological feature is lymphocytic and plasma cell infiltration of the salivary, lacrimal and other exocrine glands - bronchial, gastrointestinal, vaginal. Typically, the defeat of both large salivary glands (parotid, submandibular), and small, located in the mucous membrane of the gums and palate. At the first stages, the infiltrate is located around the small intralobular ducts, later it spreads through the parenchyma of the gland, sometimes accompanied by the formation of germinal lymphoid centers and atrophy of the glandular tissue with its replacement by adipose tissue. Characterized by proliferation and metaplasia of cells lining the ducts, with the development of the so-called myoepithelial islets, which are found in salivary gland biopsy specimens in 30-40% of cases. The general lobular structure of these glands is usually preserved; in a number of patients, some lobules are not changed, others are almost completely destroyed. The size of the affected glands can be either enlarged or normal. It is important to note that, although the clinical manifestations of Sjögren's syndrome are noted 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 found lymphocytic infiltrates in the submandibular glands in all those who died with RA.

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. Cells acquire a more "young" appearance and polymorphism, show signs of invasive spread (in particular, the structure of the lymph nodes can be completely erased). In such cases, morphologically it is not always possible to clearly distinguish between benign and malignant lymphoproliferation, in connection with which the term "pseudolymphoma" arose. In some patients, the histological picture corresponds to immunoblastic lymphadenopathy; development of true lymphosarcomas is also possible.

Symptoms of Sjögren's Syndrome:

Clinical picture:

Xerophthalmia, i.e. dryness of the eyes associated with insufficient production of tears, is usually the first sign of the disease. Patients complain of a feeling of dryness in the eyes, a sensation of a foreign body ("sand"), burning, itching, pain, discomfort when reading, difficulty opening the eyes in the morning, photophobia. On examination, the eyes appear irritated, dry, with dilated conjunctival and pericorneal vessels. A small amount of crumbly or thick viscous discharge of white or yellowish color is characteristic. In patients, lacrimation is reduced up to the complete absence of tears, which is especially noticeable with severe negative emotions (inability to cry) and exposure to irritants (in particular, the absence of lacrimation 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 1% Bengal rose solution (the results of this technique, however, are not always interpreted unambiguously) and especially with the help of a slit lamp, which makes it possible to reliably establish pinpoint and filamentous keratitis. To recognize reduced tearing, the most popular 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 thirds. Normally, the paper gets wet in 5 minutes by at least 15 mm. With lower results, the secretion of tears can be stimulated by inhalation of 10% ammonium chloride solution, the vial of which is placed 15 cm below the nose ("forced Schirmer test"). A positive Schirmer test (that is, the very fact of insufficient tearing) is not synonymous with dry keratoconjunctivitis, especially if this study is carried out at a high temperature in a dry room.

A clinically noticeable increase in the lacrimal glands in Sjögren'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 an inflammatory infiltrate, since the surgical removal of these glands (for other reasons) does not lead to such pronounced dryness. Apparently, the defeat in Sjögren's syndrome is also of all mucous glands of the conjunctival sac in the development of xerophthalmia. Perhaps a change in the biochemical composition of tears can 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 a secondary infection, which is primarily due to the weakening or absence of the bactericidal action of lysozyme contained in the normal secretion of the lacrimal glands. As a result of an infection (bacterial or viral), multiple complications are possible - fusion of the eyelids with the eyeball, loss of the blink reflex, ulcerative keratitis, corneal perforation 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 is seen 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 them. Moreover, even with indisputable histological signs of the disease, the amount of saliva sometimes remains sufficient so that the patient does not experience discomfort.

Severe xerostomia in Sjögren's syndrome can be very painful. The greatest difficulties arise when chewing and swallowing solid food, and therefore patients are forced to constantly drink it with water. Attempts to combat dryness by sucking on lemon or sour candies are only successful 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 mucous membrane of the pharynx can be the cause of dysphagia; in some cases, there was a true violation of the mobility of the esophagus.

Painful cracks in the lips and corners of the mouth are very common. The mucous membrane of the mouth is often irritated, its superficial tissue easily exfoliates, taking hot or spicy food causes pain. When milk is consumed, its coagulated threads that linger on the mucous membrane of the cheeks are sometimes incorrectly interpreted as fungal infections (which in some cases can indeed complicate xerostomia in Sjögren's syndrome). Often painful atrophic glossitis with fissures of the tongue. Ulcerative stomatitis is also possible. Normal accumulation of saliva at the base of the frenulum of the tongue in clinically expressed cases is not observed. It is also not possible to increase salivation after massage of the parotid and submandibular glands. In most patients, an increase in the parotid glands is unilateral and temporary, although both the preservation of normal sizes and bilateral enlargement are possible. The submandibular glands are less frequently enlarged. Enlarged salivary glands are often slightly tender to palpation and are usually firm, with a smooth or uneven surface. At the same time, they are not characterized by 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 the result of the often observed expansion and tortuosity of the intraglandular salivary ducts. To judge the pathology of these ducts, as well as the degree of atrophy of the parenchyma of the gland, contrast sialography can be successfully used. The severity of the destruction of the glandular parenchyma is also determined by scintigraphy using labeled technetium (99mTe). This compares the level of absorption of the radionuclide by the tissues of the salivary glands and, as a control, the thyroid gland. It is peculiar that the amount of saliva secreted per unit of time, which, it would seem, should characterize xerostomia with particular accuracy, is in fact not a sufficiently reliable indicator, since it is subject to sharp individual fluctuations. For a general judgment on the decrease in salivation, it is sometimes important to verify the small stimulating effect of acidic foods, such as lemon.

With insufficient saliva, gingivitis, bad breath, caries with toothache are frequent. Teeth easily crumble, previously placed fillings fall out of them. 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 genital organs. The apocrine glands of the female external genital organs are often affected in Sjögren's syndrome, which is accompanied by dryness and atrophy of the vaginal mucosa. Patients complain of sensations of dryness, burning and dyspareunia. Histological examination often reveals nonspecific vaginitis. The interpretation of these changes is complicated by the fact that they are more often recorded in women during menopause and therefore may be the result of estrogen deficiency, and not dry syndrome as such.

Systemic manifestations in Sjögren's syndrome often present serious difficulties in their pathogenetic interpretation. They can:

• be symptoms of specific connective tissue diseases, with which Sjögren's syndrome is very often combined;
• reflect the actual pathogenetic features of the syndrome in question
• be a non-specific consequence of insufficient function of the exocrine glands with dry mucous membranes, etc.

Skin changes. A common symptom of Sjögren's syndrome is dry skin, although patients rarely complain about it actively. In most patients, an increase in ambient temperature, exercise, or injections of pilocarpine cause noticeable sweating. However, in some cases this does not happen, and a skin biopsy reveals inflammatory changes in the area of ​​the sweat glands with the destruction of their structure. Skin. changes that are a reflection of vasculitis (ulcers, hemorrhages) are discussed below.

Urticaria is very often observed, which is a reflection of drug allergy inherent in Sjögren's syndrome. K. Whaley et al. even found that allergic reactions to penicillin in RA almost exclusively occur in patients with concomitant Sjögren's syndrome.

Damage to joints and muscles. From 50 to 70% of all patients with Sjögren's syndrome have a concomitant inflammatory connective tissue disease (usually RA), which determines the nature of the articular pathology. However, among patients with primary Sjögren's syndrome, 10-15% also have arthralgia or arthritis that does not fit into the clear framework of any specific concomitant nosology. Arthritis is usually mild and transient. Despite the possible recurrence, erosion does not occur. The reverse development of inflammatory changes occurs without treatment. The knee and elbow joints are most commonly affected. 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 Sjögren'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 the dry syndrome, reverse relationships are also possible. Therefore, at the first appearance of articular symptoms in a patient with long-term isolated Sjögren's syndrome, the onset of true RA cannot be excluded.

Severe muscle damage in primary Sjögren's syndrome almost never occurs, there are only a few relevant descriptions. Patients in some cases complain of mild myalgia and moderate muscle weakness, the latter may also be associated with electrolyte disturbances and tubular acidosis. Muscle biopsy in patients with primary Sjögren'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 with electron microscopy.

Respiratory system. Almost 50% of patients with Sjögren's syndrome note dryness of the mucous membranes of the nose. In some cases, it is significant and is accompanied by the formation of painful hard crusts and nosebleeds. Taste and olfactory sensations may be weakened or changed. On examination, atrophy of the nasal mucosa is found in many patients. Sometimes there is dryness of the vocal cords, on which there may be overlays of viscous mucus. These changes lead to a hoarse voice. A sharp decrease in hearing and serous otitis media are possible due to the closure of the nasopharyngeal opening of the auditory tube with dry crusts; removal of crusts can lead to rapid improvement.

Often the so-called dry bronchitis, in which a 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 production of IgA. Relatively specific for Sjögren's syndrome are considered infiltrates of the walls of the bronchioles and alveolar septa with T and B lymphocytes, which can clinically manifest as symptoms of airway obstruction with cough and shortness of breath, as well as episodes of usually mild interstitial pneumonia. Often described mild short-term pleurisy, self-resolved within 1-2 weeks. Chronic interstitial inflammation (fibrosing alveolitis) is relatively rare.

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

Kidney pathology. In a number of patients with Sjögren's syndrome, latent or overt signs of impaired renal tubular function were found, manifested by tubular acidosis, aminoaciduria and renal glucosuria. The mechanisms for the development of these disorders have not been fully elucidated. There is, in particular, the assumption that they are associated with chronic pyelonephritis or with increased blood viscosity as a result of hyperglobulinemia, often characteristic of Sjögren's syndrome. It is more likely that this pathology is directly related to immune responses, since relevant patients show infiltration of the medullary layer of the kidneys with lymphocytes and plasma cells. This view is supported by the fact that tubular acidosis in Sjögren's syndrome is reduced after treatment with cytostatic immunosuppressants. In addition, he draws attention to the fact 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, then against the background of 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 glomerular membrane. It is important to note that this does not mean concomitant SLE, in which the development of Sjögren's syndrome is quite real, since none of these patients had antibodies to native DNA that are highly specific for patients with lupus nephritis.

Digestive and abdominal organs. Dryness of the mucous membrane of the esophagus, which occurs with severe Sjögren's syndrome, can cause mild dysphagia in some patients when taking solid food. Often chronic atrophic gastritis with markedly reduced acidity, sometimes combined with the presence of antibodies to parietal cells. Some patients have described acute pancreatitis. Serious pathology of the intestine and violations of intestinal absorption is not observed.

A slight enlargement of the liver and spleen, in combination with mild laboratory signs of liver dysfunction, is not uncommon. Relatively often, antibodies to mitochondria, smooth muscles and liver cell membranes are found, which is considered a definite indication of the participation of the liver in the immune pathological process. The possibility of developing a specific hepatic pathology (chronic active hepatitis or primary biliary cirrhosis) as a manifestation of Sjogren's syndrome (as indicated by some authors) does not seem to be proven convincingly enough. In such cases, reverse causal relationships cannot be ruled out, since secondary Sjogren's syndrome often occurs in these autoimmune diseases (just as in RA in combination with dry syndrome, joint damage cannot be considered a manifestation of Sjogren's syndrome). Among the possible endocrine pathology, histological signs of chronic thyroiditis of the Hashimoto type should be indicated 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.

In a number of patients with severe Sjögren's syndrome, vasculitis of medium and small vessels is observed, clinically manifested 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 an immunocomplex pathology (at least in some cases). Its main symptom is "hyperglobulinemic purpura", manifested by foci of hemorrhages on the legs. Elements of hemorrhages in characteristic cases are accompanied by itching and protrude above the surface of the skin, the temperature of which is usually somewhat 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 Sjögren's syndrome. Even with primary dry syndrome, Raynaud's syndrome occurs more frequently than in the general population. However, its association with vasculitis itself seems doubtful.

CNS changes and cranial nerves by most authors are explained by vasculitis of the corresponding localization. A wide spectrum of cerebral symptoms has been described, including nystagmus, recurrent hemiparesis, and, in severe cases, serous meningoencephalitis. Among the cranial nerves, the trigeminal is relatively more often affected with the development of a characteristic pain syndrome. In isolated patients with Sjögren's syndrome, severe necrotizing arteritis was observed with clinical manifestations consistent with classic polyarteritis nodosa. However, it cannot be ruled out that polyarteritis in such cases was not a manifestation of Sjögren's syndrome, but a "comorbid" disease (like RA and others).

oncological complications. Compared with the general population, patients with Sjögren's syndrome (especially primary) are more likely to develop malignant tumors, primarily lymphosarcomas of the salivary glands. It is possible that the basis for the development of this pathology is the initial increased proliferative activity of B cells, during which a high probability of mutations is created. In combination with the weakening of cellular immune responses discussed below, this circumstance can significantly facilitate the development of tumors. Between the usual lymphoid infiltration of the salivary glands and lymphosarcoma, there is, as it were, an intermediate stage called pseudolymphoma. In appropriate patients, the salivary glands, lymph nodes and spleen are enlarged, Hyperglobulinemic purpura, a benign lymphoid infiltration of the renal tissue, develops. Although steroid therapy can reverse these changes, they are considered a premalignant condition. Such patients should be closely monitored with early biopsies, especially in cases of rapidly enlarged lymph nodes and spleen. The malignant transformation of the process, in addition to histological changes in biopsy specimens, may also be indicated by a decrease in the previously elevated level of serum globulins and the appearance of monoclonal immunoglobulins. Attention is drawn to the increased risk of developing lymphosarcomas after radiotherapy of enlarged parotid glands. In addition, with Sjögren's syndrome, there is a more frequent development of skin cancer.

Immunological characteristics of Sjögren's syndrome. A characteristic feature of dry syndrome is hyperglobulinemia. It is especially pronounced in patients with primary Sjögren's syndrome, causing in some cases clinical manifestations of hyperglobulinemic purpura and a symptom complex of increased viscosity. In most cases, the level of several or all classes of immunoglobulins is elevated. These patterns, combined with the ability of lymphocytes from salivary gland biopsies to secrete immunoglobulins in cell culture, have led to the notion of nonspecific activation of B cells in Sjögren's syndrome. This activation largely explains the high frequency of formation of the autoantibodies and immune complexes discussed below, as well as the tendency to lymphoproliferative reactions.

Diagnosis of Sjögren's Syndrome:

In almost 100% of patients, classical RF of the IgM class is detected in high titers (using the Waaler-Rose reaction), and its especially high content is characteristic not only of combinations with RA, but also of primary Sjögren's syndrome. Levels of rheumatoid factors belonging to the IgG and IgA classes are also elevated in the blood and saliva. It is very likely that rheumatoid factors are largely responsible for the formation of circulating immune complexes, the number of which is increased in most patients with both variants of Sjögren's syndrome. A number of manifestations of this disease correspond to the recognized ideas about the immunocomplex pathology (vasculitis, arthritis, arthralgia, as well as rare interstitial nephritis and glomerulonephritis). However, there is no concrete evidence of a pathogenic role for immune complexes in Sjögren's syndrome; in any case, their level does not reflect the activity of the disease.

Quite often, antibodies to components of cell nuclei are found. Their total determination by immunofluorescence gives a positive result in almost 90% of patients with primary Sjögren's syndrome and in almost half of patients with secondary. The nature of nuclear fluorescence is usually diffuse or spotty, less often nucleolar. Much attention is paid to the determination of antibodies to specific complex antigens, which most likely have the character of ribonucleoproteins. The most important among them are the antigens Ro (apparently cytoplasmic) and La (having both cytoplasmic and nuclear origin). Neither the functional role of these soluble antigens nor their precise localization in the cell has been established yet.

Anti-Ro antibodies are found in half of all patients with Sjögren's syndrome; reports on the frequency of detection of anti-La antibodies are more contradictory (according to different authors, from 10 to 80%). Both antibodies are most characteristic of primary Sjögren's syndrome, as well as secondary, combined with SLE. It is noteworthy that in these patients a clear correlation of anti-Co antibodies with vasculitis was established. D. Isenberg et al. noted that the detection of anti-La antibodies in patients with polyarthralgia indicates a greater likelihood of subsequent development of Sjögren's syndrome than RA. It has been suggested that there is an immunogenetic association of HLAB8 and DR3 relatively often found in primary dry syndrome with antibodies to Ro and La antigens. It should be borne in mind that these antibodies are found not only in patients with Sjögren's syndrome, but also in SLE with predominantly skin manifestations (subacute cutaneous lupus erythematosus), as well as in sick children and their mothers with a rare neonatal lupus syndrome.

Antibodies to native DNA and deoxyribonucleoproteins, so characteristic of SLE, are found in approximately 20% of patients with primary Sjögren's syndrome in low or moderate titers. At the same time, their presence and level do not reflect any organ changes. Antibodies to the Golgi complex and to polyphosphoribose have also been described, but their significance has not been established.

Organ-specific and tissue-specific antibodies in Sjögren's syndrome are found in almost 1/3 of patients. These include antibodies to the pancreas and thyroid glands, ovaries, smooth and skeletal muscles, liver cell membranes and lining (parietal) cells of the stomach. Their clinical significance is uncertain; the only exception can be considered a relatively natural combination of a high titer of antibodies to thyroid microsomes and thyroglobulin with signs of thyroiditis. Antibodies to the tissues of the ducts of the salivary glands are much more often found in the combination of RA with Sjögren's syndrome (in 50-70% of patients) than in "normal" RA (20-30%) or in primary Sjögren's syndrome (10-20%). An inverse correlation is observed between the presence of these antibodies and the severity of salivary gland lesions. Thus, there are serious grounds to believe that this type of antibody is either a secondary phenomenon that has no clinical significance, or plays a protective role by blocking the antigenic determinants of downstream cells.

The above manifestations of increased activity of B cells in Sjögren's syndrome (hyperglobulinemia, autoantibodies, immune complexes) are possibly the result of a decrease in the suppressive effect of T lymphocytes. Indeed, a decrease in the number of T-suppressors has been noted in peripheral blood and in lip biopsies of patients with classic dry syndrome. Thus, pronounced humoral disorders may be secondary to pathology in the cellular link of immunity. Specific examples of a decrease in cellular immune responses in patients with primary Sjögren's syndrome are a decrease in the severity of lymphocyte blast transformation and skin reactions to intradermal administration of standard antigens (tuberculin, etc.).

The basis for the diagnosis of Sjögren's syndrome is a statement in a patient of two of the following three criteria: xerophthalmia, xerostomia, the presence of RA or another diffuse connective tissue disease. Very rarely, chronic aggressive hepatitis, primary biliary cirrhosis, or sarcoidosis may be present as a concomitant disease. Often, however, errors in the recognition of xerophthalmia and xerostomia. Sometimes patients exaggerately characterize their sensations as dryness of the eyes and mouth, and in such cases, targeted questioning and examination make it possible to immediately verify the abundant secretion of saliva and tears. Very useful in this regard are simple questions about the ability to swallow dry food without drinking liquid, the presence of tears during difficult experiences or cutting onions, etc. The manifestations of true xerostomia and xerophthalmia are described in the "Clinical picture" section.

The question of Sjögren's syndrome arises not only when patients complain of dryness, but also with an increase in the salivary glands, especially the parotid glands. In such situations, the doctor must keep in mind that their increase is characteristic, in addition to dry syndrome, also of tumor pathology (primary benign and malignant tumors of the salivary glands, lymphosarcomas, Waldenström's macroglobulinemia), inflammatory diseases (sarcoidosis, acute bacterial and viral infections of the glands, chronic sialadenitis , tuberculosis, syphilis, actinomycosis, histoplasmosis, hookworm) and a number of other diseases (allergy to iodine, lead and copper compounds, salivary duct stones, diabetes mellitus, liver cirrhosis, hyperlipidemia, starvation).

Tumors should especially be thought of with unilateral progressive enlargement of the gland, its high density and the absence of other signs of dry syndrome. In favor of Sjögren's syndrome, fluctuations in the size of the glands, the absence of pain in them, a chronic enlargement of the glands without involving the surrounding tissues in the process, testify.

It must be remembered that an increase in the salivary and lacrimal glands can occur with lymphosarcoma, lymphocytic leukemia and sarcoidosis, but dry syndrome among the listed diseases is possible almost exclusively with sarcoidosis. In doubtful cases, a biopsy of one of the minor salivary glands can be used to confirm the diagnosis of Sjögren's syndrome. A biopsy of a large gland is justified only if a tumor disease is suspected. Most of the other diseases listed above, with a correct assessment of their clinical picture and anamnesis of patients, can be quite clearly differentiated from Sjögren's syndrome (primarily by the absence of xerophthalmia and xerostomia).

For correct diagnostic generalization in rare severe cases of primary Sjögren's syndrome, it is necessary to take into account that the clinical manifestations of this disease, in addition to xerostomia and xerophthalmia, may include the following syndromes: vasculitis (panarteritis, necrotizing arteritis); hyperglobulinemic purpura; lymphoproliferation and related diseases; hepatosplenomegaly; enlargement of the parotid salivary glands; fibrosing alveolitis and signs of obstructive airway disease; Raynaud's syndrome; renal tubular acidosis; glomerulonephritis; polyneuritis; anemia, leukopenia, thrombocytopenia; myositis.

A correct understanding of the possibility of these syndromes is important for the correct diagnosis within a single nosological unit, without resorting to the assumption of a combination of several diseases. It should be noted once again that the above systemic pathology, especially in the aggregate, is relatively rare in primary Sjögren's syndrome. To an even lesser extent, it is characteristic of patients with secondary Sjögren's syndrome (if we mean clinical symptoms that do not serve as a manifestation of a concomitant connective tissue disease).

Important for establishing the diagnosis of Sjögren's syndrome is the detection in almost all patients with RF, and in a number of patients in the highest possible titers. In primary dry syndrome, antibodies to the Ro and La antigens are often found.

Treatment of Sjögren's Syndrome:

Treatment of patients with Sjögren's syndrome usually consists in the treatment of both manifestations of the dry syndrome itself and a concomitant systemic disease (RA, SLE, etc.). The treatment of concomitant diseases is carried out without any fundamental features, however, doctors need to keep in mind the slightly greater tendency of patients with Sjögren's syndrome to allergic reactions to drugs. Therefore, the appointment of the latter must be strictly justified. As a rule, after discharge from the hospital, patients should be under the supervision of a rheumatologist, ophthalmologist and dentist. It is necessary to pay special attention to the possible increase in the size of the salivary glands and the appearance of signs of lymphoproliferation in order to conduct an appropriate oncological examination.

For the treatment of dry syndrome as such, symptomatic agents are mainly used that significantly improve the condition and well-being of many patients. At the same time, it is undesirable to prescribe antihistamines and antidepressants, which can significantly increase the dryness of the mucous membranes.

With dry keratoconjunctivitis, at least half of the patients receive significant relief from the instillation of "artificial tears" into the eye, the main component of which is usually a 0.5% solution of methylcellulose. Since, in addition to dryness, in some cases the accumulation of viscous mucus causes great inconvenience, such patients should use a 5-10% solution of the mucolytic drug acetylcysteine ​​in the form of eye drops. The frequency of instillation of these drugs is determined individually. Some authors note the effectiveness of the use of soft contact lenses, which slow down the evaporation of tears from the eyes. While maintaining a small lacrimation, the closure of the nasolacrimal ducts by electrocoagulation is successfully used. In cases of secondary bacterial or fungal infection of the eye, vigorous treatment with appropriate antibiotics is indicated.

Xerostomia, especially when severe, is difficult to treat. The jelly-like lubricants used for this purpose proved to be ineffective and in some cases increased the sensation of dry mouth. Therefore, the best method of combating xerostomia remains the use of water or other liquids, which makes it easier to chew and free the oral cavity from food debris. Careful dental care and regular dental consultations are necessary to actively treat the first symptoms of complications (gingivitis, caries, thrush, etc.). With rare purulent parotitis that does not respond to antibiotic treatment, as well as with complete blockage of the salivary duct by a stone, surgical intervention is necessary.

Dryness in the nose is relieved by irrigation with isotonic sodium chloride solution. Intranasal administration of lubricants containing various oils is undesirable due to the risk of developing lipoid pneumonia. Dry skin rarely requires special treatment, and emollient creams are usually sufficient. With dryness of the vagina, emollient gels are prescribed.

Attempts have also been made to reduce the dryness of the mucous membranes with the help of not local, but general action, in particular bromhexine in a daily dose of 24-48 mg. A controlled randomized trial showed that the administration of this drug led to an increase in the secretion of tears, but not saliva, which was the main calculation.

In patients with primary Sjögren's syndrome, in addition to the methods of local treatment of dry mucous membranes discussed above, if necessary, drugs of a different effect are also used: alkalis for renal tubular acidosis, NSAIDs for arthralgia and arthritis, nifedipine for Raynaud's syndrome. In cases of severe myositis, rare hemolytic anemia, or severe systemic vasculitis with neurological complications, moderate daily doses of corticosteroids (20-40 mg prednisolone) are indicated, with the usual gradual dose reduction to minimal maintenance and subsequent withdrawal. With the development of lymphoproliferative syndrome (enlarged lymph nodes and spleen, often in combination with an increase in the size of the salivary glands and cryoglobulinemic purpura and pulmonary infiltration), corticosteroids are also prescribed (starting with 30-40 mg of prednisolone per day), often in combination with cyclophosphamide or azathioprine. Local radiotherapy of enlarged salivary glands in Sjögren's syndrome is contraindicated due to the risk of lymphosarcoma. Prescribing prednisone solely for salivary or lacrimal gland enlargement is also not recommended, since the dryness of the mucous membranes as a result of this treatment is not reduced, despite the possible reduction in the size of the spleens. There are reports of positive results in the treatment of deer with Sjögren's syndrome with cyclophosphamide alone, but they are few and therefore need to be confirmed.

In recent years, data have appeared on the successful use of pulse therapy in severe variants of Sjögren's syndrome with methylfednisolone (3 days in a row at 1000 mg / day slowly intravenously) or methylprednisolone in combination with cyclophosphamide (the latter at a dose of 1000 mg is administered additionally on the first day of therapy). As a result, the signs of xerostomia 4 xerophthalmia decreased, there was a clinical improvement in systemic manifestations and a positive dynamics of immunological parameters. The authors believe that pulse therapy can be considered the method of choice for such manifestations of Sjögren's syndrome as vasculitis with necrotizing ulcerative skin changes, polyneuritis, hyperglobulinemic (cryoglobulinemic) purpura, exudative polyserositis, cerebrovasculitis, and autoimmune hemolytic anemia. According to the observations of the same authors, the results of pulse therapy increase even more when it is combined with plasmapheresis or hemosorption.

Which doctors should you contact if you have Sjögren's Syndrome:

Rheumatologist

Are you worried about something? Do you want to know more detailed information about Sjögren's Syndrome, its causes, symptoms, methods of treatment and prevention, the course of the disease and diet after it? Or do you need an inspection? You can book an appointment with a doctor– clinic Eurolaboratory always at your service! The best doctors will examine you, study the external signs and help identify the disease by symptoms, advise you and provide the necessary assistance and make a diagnosis. you also can call a doctor at home. Clinic Eurolaboratory open for you around the clock.

How to contact the clinic:
Phone of our clinic in Kyiv: (+38 044) 206-20-00 (multichannel). The secretary of the clinic will select a convenient day and hour for you to visit the doctor. Our coordinates and directions are indicated. Look in more detail about all the services of the clinic on her.

(+38 044) 206-20-00

If you have previously performed any research, be sure to take their results to a consultation with a doctor. If the studies have not been completed, we will do everything necessary in our clinic or with our colleagues in other clinics.

You? You need to be very careful about your overall health. People don't pay enough attention disease symptoms and do not realize that these diseases can be life-threatening. There are many diseases that at first do not manifest themselves in our body, but in the end it turns out that, unfortunately, it is too late to treat them. Each disease has its own specific signs, characteristic external manifestations - the so-called disease symptoms. Identifying symptoms is the first step in diagnosing diseases in general. To do this, you just need to several times a year be examined by a doctor not only to prevent a terrible disease, but also to maintain a healthy spirit in the body and the body as a whole.

If you want to ask a doctor a question, use the online consultation section, perhaps you will find answers to your questions there and read self care tips. If you are interested in reviews about clinics and doctors, try to find the information you need in the section. Also register on the medical portal Eurolaboratory to be constantly up to date with the latest news and information updates on the site, which will be automatically sent to you by mail.

Sjögren's disease is a systemic autoimmune disease related to connective tissue diseases; characterized by the defeat of many secreting glands, mainly salivary and lacrimal.

There is also Sjögren's syndrome (damage to the lacrimal and salivary glands), which accompanies rheumatoid arthritis, diffuse connective tissue diseases, diseases of the biliary system and other autoimmune diseases.

Sjögren's disease is the most common disease among connective tissue diseases and occurs in women 10–25 times more often than in men, usually at the age of 20–60 years, and is much less common in children.

The causes of the disease are unknown. Most researchers consider Sjögren's disease as a consequence of immunopathological reactions to a viral infection, presumably retroviral.

Manifestations of Sjögren's disease

Symptoms of Sjögren's disease can be divided into glandular and extraglandular.

Glandular manifestations are due to damage to the secreting glands, characterized mainly by a decrease in their function.

A constant sign of damage to the lacrimal glands in Sjögren's disease is eye damage associated with a decrease in the secretion of lacrimal fluid. Patients complain of burning sensations, “scratches” and “sand” in the eyes. Often there is itching of the eyelids, redness, accumulation in the corners of a viscous white discharge. Later, photophobia, narrowing of the palpebral fissures appear, visual acuity decreases. An increase in the lacrimal glands in Sjögren's disease is rare.

The second obligatory and constant sign of Sjögren's disease is the defeat of the salivary glands with the development of chronic inflammation. It is characterized by dry mouth and enlarged salivary glands.

Often, even before the appearance of these signs, dryness of the red border of the lips, seizures, stomatitis, enlargement of nearby lymph nodes, multiple (often cervical) dental caries are noted. In a third of patients, a gradual increase in the parotid glands is observed, leading to a characteristic change in the oval of the face, described in the literature as a “hamster muzzle” or “chipmunk muzzle”.

In the initial stage of the disease, dry mouth appears only with physical exertion and excitement.

In the advanced stage, dry mouth becomes permanent, accompanied by the need to drink dry food, the desire to moisten the mouth while talking. The mucous membrane of the oral cavity becomes bright pink, easily injured. There is little free saliva, it is foamy or viscous. Dry tongue. The lips are covered with crusts, inflammation is noted, a secondary infection, including fungal and viral, can join. Characterized by multiple cervical caries of the teeth.

The late stage is manifested by a sharp dryness of the oral cavity, the inability to speak, swallow food without drinking it with liquid. The lips in such patients are dry, cracked, the mucous membrane of the oral cavity with keratinization, the tongue is folded, free saliva in the oral cavity is not determined.

Dryness of the nasopharynx with the formation of dry crusts in the nose, in the lumen of the auditory tubes can lead to temporary deafness and the development of otitis. Dryness of the pharynx, as well as vocal cords, causes hoarseness of the voice.

Frequent complications are secondary infections: sinusitis, recurrent tracheobronchitis and pneumonia. The defeat of the glands of the external genital organs is observed in approximately 1/3 of patients with Sjögren's disease. The mucous membrane of the vagina is reddened, dry, often patients are disturbed by burning pains and itching.

Dry skin is a common symptom of Sjögren's disease. Sweating may be reduced.

Violation of swallowing is due to the presence of dry mucous membranes. Many patients develop chronic atrophic gastritis with severe secretory insufficiency, clinically manifested by heaviness and discomfort in the epigastric region after eating, belching with air, nausea, and loss of appetite. Rarely observed pain in the epigastric region.

There is a direct relationship between the degree of dryness and inhibition of the secretory function of the stomach. The defeat of the biliary tract (cholecystitis) and liver (hepatitis) is observed in most patients. There are complaints of heaviness and pain in the right hypochondrium, bitterness in the mouth, nausea, poor tolerance to fatty foods.

Involvement of the pancreas (pancreatitis) is manifested by pain and indigestion.

Extra-glandular manifestations of Sjögren's disease are very diverse and systemic. Pain in the joints, slight stiffness in the morning. Signs of muscle inflammation (muscle pain, moderate muscle weakness, a slight increase in the level of creatine phosphokinase in the blood) are observed in 5-10% of patients.

In most patients with Sjögren's disease, there is an increase in submandibular, cervical, occipital, supraclavicular lymph nodes, and in 1/3 of patients, an increase in lymph nodes is common. In the latter case, an increase in the liver is often detected.

Various respiratory tract lesions are observed in 50% of patients. Dry throat, itching and scratching, dry cough and shortness of breath are the most common complaints.

In Sjögren's disease, vascular damage is noted. Spotted bloody rashes often appear on the skin of the legs, but over time they spread higher and can be found on the skin of the thighs, buttocks and abdomen. Rashes are accompanied by itching, painful burning and an increase in skin temperature in the affected area.

The defeat of the nervous system with impaired sensitivity of the type of "socks" and "gloves", neuritis of the facial and trigeminal nerves are observed in a third of patients.

A third of patients have allergic reactions, more often - to antibiotics, sulfonamides, novocaine, group B drugs, as well as to chemicals (washing powders, etc.) and food products.

Diagnostics

The most informative laboratory indicators for Sjögren's disease are high ESR, a decrease in the number of leukocytes, hypergammaglobulinemia (80–70%), the presence of antinuclear and rheumatoid factors (90–100%), as well as antibodies to soluble nuclear antigens SS-A/Ro and SS- B/La (60–100%). A third of patients have cryoglobulins.

In polyclinic conditions, it is advisable to take into account various combinations of the following features:

• joint damage;
• inflammation of the parotid salivary glands and gradual enlargement of the parotid glands;
• dryness of the oral mucosa (nasopharynx) and the rapid development of multiple, predominantly cervical, dental caries;
• recurrent chronic conjunctivitis;
• persistent increase in ESR (over 30 mm / h);
• hypergammaglobulinemia (over 20%);
• the presence of rheumatoid factor in the blood (titer more than 1: 80).

Although none of these signs, taken separately, is specific for Sjögren's disease, the presence of four signs or more makes it possible to suspect in 80–70% of cases and subsequently confirm the diagnosis using special research methods.

The differential diagnosis for Sjogren's disease is carried out with rheumatoid arthritis, systemic lupus erythematosus, autoimmune diseases of the liver and biliary tract in combination with Sjogren's syndrome.

Treatment of Sjögren's disease

The main place in the treatment of Sjögren's disease belongs to hormones and cytostatic immunosuppressants (chlorbutin, cyclophosphamide).

In the initial stage of the disease, in the absence of signs of systemic manifestations and moderate violations of laboratory parameters, long-term treatment with low doses of prednisolone (5-10 mg / day) is advisable.

In the severe and late stages of Sjögren's disease, in the absence of signs of systemic manifestations, it is necessary to prescribe prednisolone (5-10 mg / day) and chlorbutin (2-4 mg / day), followed by long-term, for several years, taking maintenance doses of prednisolone (5 mg / day). day) and chlorbutin (6–14 mg/week).

Such a scheme can be used to treat patients in the initial stages of the disease in the presence of severe violations of laboratory indicators of process activity, as well as cryoglobulinemia without clear signs of systemic manifestations.

Pulse therapy with high doses of prednisolone and cyclophosphamide (1000 mg of 6-methylprednisolone intravenously daily for three consecutive days and a single intravenous injection of 1000 mg of cyclophosphamide) followed by a transition to moderate doses of prednisolone (30–40 mg/day) and cytostatics (chlorbutin 4- 6 mg/day or cyclophosphamide 200 mg intramuscularly once or twice a week) in the absence of liver effects is the most effective treatment for patients with severe systemic manifestations of Sjögren's disease, is generally well tolerated by patients and avoids many complications associated with long-term use high doses of prednisolone and cytostatics.

Extracorporeal methods of treatment (hemosorption, cryoadsorption, plasmapheresis, double plasma filtration) in combination with pulse therapy are the most effective in the treatment of patients with Sjögren's disease with ulcerative necrotic vasculitis, glomerulonephritis, polyneuritis, myelopolyradiculoneuritis, cerebrovasculitis caused by cryoglobulinemia.

Local therapy for eye damage is aimed at eliminating dryness, preventing secondary infection. Dry eyes are an indication for artificial tears. The frequency of use of drugs depends on the severity of damage to the organ of vision and ranges from 3 to 10 times a day.

Medical soft contact lenses are used to protect the cornea. For the prevention of secondary infection, solutions of furacilin (at a dilution of 1: 5000), a 0.25% solution of levomycetin, ciprofloxacin, etc. are used.

Therapy of chronic inflammation of the salivary glands is aimed at overcoming dryness, strengthening the walls of the ducts of the salivary glands, preventing exacerbations, improving the restoration of the epithelium of the oral mucosa and combating secondary infection.

In order to normalize nutrition and secretion of the salivary glands, novocaine blockades are used. In cases of exacerbation of chronic parotitis (and for the prevention of its recurrence), applications of a 10–30% solution of dimexide are used. In cases of development of purulent parotitis, antibiotics are injected into the ducts of the salivary glands and antifungal drugs (nystatin, levorin, nystatin ointment) are locally prescribed. To reduce the permeability of the ducts, calcium preparations are administered intravenously or intramuscularly.

It is possible to accelerate the healing of the oral mucosa and the red border of the lips in the event of erosion and cracks with the help of rosehip and sea buckthorn oils, solcoseryl and methyluracil ointments, as well as by treating the oral mucosa with ENCAD (active derivatives of nucleic acids). Decamine caramel also has antibacterial properties.

With dryness of the nasal mucosa, frequent applications of isotonic sodium chloride solution (with the help of turundas) are used.

Vaginal dryness is relieved by the use of potassium iodide jelly.

Forecast

The prognosis for life is favorable. With timely treatment, it is possible to slow down the progression of the disease, restore the ability of patients to work. With a late start of treatment, severe manifestations of the disease usually develop rapidly, and the patient becomes disabled.

Treatment of Sjögren's syndrome

Causes of Sjögren's Syndrome

A systemic autoimmune disease characterized by a chronic inflammatory process in the epithelial glands with damage to the lacrimal and salivary glands, the gradual development of their secretory insufficiency (the so-called "dry syndrome"), as well as various systemic manifestations.

Sjogren's syndrome is a lesion of the lacrimal and salivary glands with the development and which is accompanied by rheumatoid arthritis, systemic lupus erythematosus (SLE), systemic scleroderma (SSD), chronic autoimmune hepatitis, primary biliary cirrhosis and other autoimmune diseases. It is customary to distinguish between primary and secondary Sjögren's syndrome.

The disease occurs predominantly in middle-aged women, the ratio of affected women to men exceeds 9:1. The prevalence of Sjögren's disease in the general population ranges from 0.1-0.8%, and among people over 50 years of age - about 3%. The incidence of Sjögren's syndrome in rheumatoid arthritis is 15-30%, SJS - up to 20%, SLE - 10%, primary biliary cirrhosis and chronic autoimmune hepatitis - 30-80%. Patients with "dry syndrome" often show a combination of Sjogren's syndrome with rheumatoid arthritis (about 50%), in other cases - a combination of Sjogren's syndrome with other systemic diseases of the connective tissue, polymyositis, chronic autoimmune diseases of the liver, thyroid gland.

The etiology of the disease is unknown. A likely etiological factor is considered to be a chronic viral infection, which may indicate the detection of virus-like particles, HBs antigen, Epstein-Barr virus in the epithelium of the salivary glands, and an increased content of antibodies to retroviruses and viruses of the herperic group in the blood of patients.

The genetic predisposition to Sjögren's disease is evidenced by the frequent carriage of certain HLA antigens (B8, DR3, Dw3, DRw52) and an increased incidence among blood relatives of patients.

The main pathogenetic link is the development of autoimmune reactions with the formation of both organ autoantibodies to the cells of the ducts of the lacrimal, salivary and other exocrine glands (pancreas, parietal cells of the stomach, etc.), and organ-nonspecific autoantibodies - RF, ANF, antibodies to Ro antigens (SS-A ) and La (SS-B). This process is induced by environmental factors, rather by viruses, which cause the expression of the HLA-DR antigen and the translocation of autoantigens on the membranes of epithelial cells, as a result of which the latter acquire their own antigenic properties.

Hyperproduction of autoantibodies leads to the formation of a large amount of CIC, which play an important role in the development of systemic manifestations of the disease. In 30% of patients, lymphoid infiltration spreads to other tissues and organs, as a result of which the activity of the lungs, kidneys, blood vessels, muscles, and digestive tract is disrupted. The defeat of the exocrine glands and systemic manifestations are associated with poly- and oligoclonal activation of B-cells. With monoclonal B-cell activation, the risk of developing malignant lymphomas is high.

In the early stages of the disease, the salivary, lacrimal, and other exocrine glands may increase in size due to an inflammatory process with marked infiltration of lymphocytes and plasma cells, concentrating mainly around the ducts. In the future, infiltration spreads to the parenchyma of the gland with the development of destructive changes in the acini, their atrophy with replacement with adipose and fibrous tissue, and the gradual formation of secretory insufficiency. At the same time, the stroma and partial structure of the glands are slightly disturbed.

In the development of atrophy of secreting cells, not only inflammatory changes are important, but also an increase in pressure due to narrowing and obliteration of the lumen of the ducts. At first, this occurs due to infiltrative edema of the walls, and later - as a result of the proliferation of epithelial cells of the ducts and fibrosis.

Characteristic of Sjögren's disease is the formation of myoepithelial islets - foci of proliferation and metaplasia of the epithelium of the ducts surrounded by lymphocytes. In the later stages of the pathological process, iron decreases in size, its normal architectonics is disturbed. The histological picture is dominated by fibrotic changes with lymphoid follicles and myoepithelial islets. Pathological changes are found not only in large (iliac, submandibular), but also in small salivary glands, mucous membrane of the gums and palate, glands of the bronchi, stomach, genitals, sweat glands. In some patients, lymphocytic infiltrates in the exocrine glands, internal organs and lymph nodes lose their classic benign character: the cells become polymorphic, signs of invasive spread are found, the normal structure of the lymph nodes and glandular tissue is disturbed. Such a histological picture is referred to as "pseudolymphoma". Under the influence of treatment with glucocorticoids, such changes are reversed. However, it should be remembered that patients with Sjögren's disease have an increased risk of developing malignant lymphomas.

The classification of Sjögren's disease is made according to several criteria and is presented as follows:

• with the flow:
  • subacute,
  • chronic;
• by stage of development:
  • initial,
  • expressed,
  • late;
• according to the degree of activity:
  • minimum
  • moderate,
  • high.

Clinical manifestations of Sjögren's disease consist of signs of damage to the exocrine glands and systemic changes. The main clinical symptoms are associated with damage to the lacrimal and salivary glands. More often, the first sign of the disease is dry eyes - xerophthalmia, associated with insufficient production of tears. It is found in all patients.

Typical complaints are itching, a feeling of dryness, "sand in the eyes", burning and pain in the eyeballs with visual strain. These discomforts are aggravated by the end of the day due to the evaporation of moisture from the surface of the eyes during the time they were open. A decrease in lacrimation up to the complete absence of tears is especially noticeable in the case of negative emotions (patients cannot cry) and when exposed to irritating substances.

On examination, signs of irritation are found, the conjunctival and pericorneal vessels are dilated, the edges of the eyelids are thinned and hyperemic. In the corners of the eyes, a small amount of thick, viscous white or yellowish secretion may accumulate, which has a muco-filamentous structure due to desquamated corneal epithelial cells and mucin filaments. In severe cases, photophobia and decreased visual acuity occur.

Due to the weakening of the bactericidal action of lysozyme contained in tears, the tendency to secondary infection increases. In case of infection, severe complications are possible - fusion of the eyelids with the eyeball, ulcerative keratitis, corneal perforation with the threat of uveitis, secondary glaucoma and loss of vision.

The second important, although less specific, symptom is xerostomia. Reduced saliva production is a consequence of chronic parenchymal sialadenitis. Dry mouth for a long time may be insignificant, patients get used to it. Even in the presence of histological signs of sialadenitis, the amount of saliva may remain sufficient for quite a long time so as not to cause obvious discomfort. Sometimes patients pay attention to the fact that after stimulation (chewing a lemon, eating acidic foods), a large amount of thick (stagnant) saliva is released. In typical cases, signs of xerostomia first appear sporadically - with excitement, during a long conversation, and eventually become permanent. The patient is forced to drink liquid during a conversation, a night's sleep, and the consumption of dry food. Hot and spicy foods cause burning and pain of the oral mucosa. Discomfort and dryness in the mouth are so pronounced that it is difficult to chew, form a food bolus and swallow. Taste sensations may change.

Characterized by early development and rapid progression of multiple cervical caries. On examination, the mucous membrane of the oral cavity is bright pink, edematous, easily injured, there is little free saliva, it is foamy or viscous. The tongue is dry, smooth, bright red, the red border of the lips is dry, covered with crusts, cracks and inflammation are found in the corners of the mouth (angular stomatitis). Often a secondary infection joins and fungal develops, less often bacterial or viral stomatitis.

At a late stage of the disease, saliva is practically not secreted, the patient loses the ability to talk and eat without moistening the oral cavity. There are keratosis of the mucous membrane, atrophy of the papillae of the tongue, teeth fall out.

An important sign of chronic sialadenitis is an increase in the parotid salivary glands. It is found in 60% of patients. This increase can be bilateral, unilateral, or asymmetrical. In the case of a slow increase, palpation of the glands is painless or slightly painful. In half of patients with parotitis, it has a recurrent course, without exacerbation, an increase in the glands may be imperceptible. Relapses of parotitis are accompanied by swelling and soreness of the glands, complicated opening of the mouth, trigeminal sensory neuropathy.

Acute pain in the glands, local hyperemia and hyperthermia, high fever, discharge of pus from the ducts of the glands indicate the addition of a secondary infection. This is usually the result of a violation of the outflow of saliva due to tortuosity, uneven expansion, swelling of the walls of the salivary ducts inside the gland. At least 10% of patients with sialadenitis diagnosed using instrumental methods do not show glandular enlargement. The submandibular and sublingual salivary glands increase less frequently. For Sjögren's syndrome, an increase in the salivary glands and obvious clinical signs of recurrence of parotitis are not characteristic.

The development of dry (atrophic) tracheobronchitis is characteristic, in which thick mucus causes a strong dry cough, sometimes with bronchospasm. Often signs of Sjögren's disease are dryness and flaking of the skin, hyperkeratosis, and decreased sweating. The defeat of the apocrine glands of the female external genital organs in 30% of patients is accompanied by dryness and atrophy of the vaginal mucosa. In women during menopause, involutive changes due to estrogen deficiency are superimposed on the phenomena of atrophic colpitis. Perhaps the development of chronic gastritis with secretory insufficiency, up to achilia, a decrease in the exocrine function of the pancreas. However, in a significant number of patients, hypofunction of these glands is not accompanied by clinical symptoms.

Systemic manifestations of Sjögren's disease are of secondary importance for diagnosis, although they are quite common and sometimes determine the prognosis. Articular syndrome is observed in two-thirds of patients. It has a benign course. More often there are episodic arthralgias with slight morning stiffness, which disappear on their own or after taking NSAIDs.

Vascular involvement may present with Raynaud's syndrome (up to 40% of patients) or purpura (5-10% of patients). In the occurrence of Raynaud's syndrome, increased blood viscosity plays an important role, and the contribution of vasculitis is insignificant. Therefore, attacks of peripheral vasospasm are often non-classical (1-2-phase), and a severe course with the formation of ulcers and necrosis is extremely rare. Recurrent hemorrhagic rash is also associated with hyper-beta-globulinemia and has orthostatic features: small punctate hemorrhages, petechiae appear, rise from the feet to the knees, to the hips and abdomen. With prolonged purpura, hyperpigmentation of the skin of the hemosiderin type remains. Very rarely, cryoglobulinemic purpura develops with ulcerative necrotic vasculitis of the skin, accompanied by damage to the cerebral vessels, polymyeloradiculoneuritis, hepatitis, and can determine the life prognosis. Sometimes the first manifestation of Sjögren's disease may be urticarial vasculitis.

Among the diseases of the digestive tract, the most common (about 30% of patients) is dysphagia - discomfort when eating solid food. It is associated with both difficulty swallowing due to xerostomia and dryness of the esophageal mucosa, and hypotonia of the esophagus. Gastric dyspepsia and colitis symptoms are common but rarely severe.

With cryoglobulinemia, hepatitis naturally develops. A slight increase in the liver in combination with moderate laboratory signs of impaired function is more often due to the hepatotoxic effect of drugs used in the treatment of Sjögren's disease. In 20% of patients, lymphadenopathy is detected - regional (increased submandibular, cervical, supraclavicular nodes) or generalized. With a targeted x-ray and functional examination, almost half of the patients can find symptoms of interstitial pneumonia, but clinically this pathology is quite mild, cough and shortness of breath usually occur episodically. Recurrent dry or effusion pleurisy with little exudate usually resolves quickly and does not require treatment. Interstitial pulmonary fibrosis (fibrosive alveolitis) develops very rarely.

Kidney damage is observed in 10-30% of patients. This is mainly a tubular pathology - tubular acidosis, aminaciduria, phosphaturia, developing due to lymphoplasmacytic infiltration of the epithelium of the renal tubules. Some patients develop nephrogenic diabetes insipidus, chronic renal failure. Very rarely, diffuse membranous glomerulonephritis occurs. Severe forms of glomerulonephritis are observed in patients with cryoglobulinemia.

There is no specific cardiac pathology for Sjögren's disease. Very rarely, asymptomatic pericarditis and left ventricular diastolic dysfunction are diagnosed.

For patients with Sjögren's disease, the typical development of allergic reactions to many drugs and foods. With Sjögren's syndrome, the nature of the damage to organs and systems is determined by the underlying disease.

How to treat Sjögren's syndrome?

In the initial stage of Sjögren's disease, in the absence of systemic manifestations, 5-10 mg of prednisolone is prescribed in combination with aminoquinoline drugs (plaquenil or delagil).

In patients with systemic lesions, a combination of glucocorticoids with high doses of chlorbutin or cyclophosphamide is used. The dose of prednisolone, depending on the degree of disease activity, is 10-40-60 mg per day. After achieving remission, the maintenance dose of prednisolone does not exceed 5 mg per day.

The drug of choice for the basic therapy of Sjögren's disease is chlorbutin, which is used for several years: during the first year - 2-4 mg per day, in the second year - at the same dose every other day, in the third - after 2 days, in the future - twice a week. The effectiveness of other cytostatic immunosuppressants in Sjögren's disease (methotrexate, azathioprine, cyclosporine A) has not been proven.

Persons with a crisis course of Sjögren's disease and the development of generalized vasculitis or massive lymphoplasmacytic infiltration of organs and tissues are prescribed combined pulse therapy: methylprednisolone in isotonic sodium chloride solution or glucose solution for 3 consecutive days, on the 2nd day, cyclophosphamide is added to a dropper with methylprednisolone. The main indications for pulse therapy are ulcerative necrotic vasculitis, damage to the peripheral and central nervous system, immunocomplex cryoglobulinemic nephritis, prolonged massive enlargement of the salivary glands with biopsy-proven severe lymphoid infiltration and the synthesis of monoclonal immunoglobulins, pseudolymphoma and some types of benign lymphomas, lymphoid infiltration of the lungs and alveolar pulmonary fibrosis. With autoimmune hemolytic anemia and thrombocytopenia, classical pulse therapy is performed without the addition of cyclophosphamide. In especially severe cases, pulse therapy can be combined with efferent therapy methods - plasmapheresis, cryopheresis, double plasma filtration.

Indications for efferent therapy include necrotizing ulcerative vasculitis, polyneuritis and central nervous system involvement, cryoglobulinemic nephritis, cryoglobulinemia with monoclonal hyper-β-globulinemia. Evidence of the effectiveness of anticytokine drugs in Sjögren's disease is not yet available.

In the presence of xerophthalmia, replacement therapy is constantly carried out: instillation into the eyes of the so-called artificial tears (for example, methylcellulose), other hydrophilic polymers (for example, polyvinylpyrrolidone) from 3 to 8 times a day. The accumulation of viscous mucus is prevented by instillation of a 5-10% solution of acetylcysteine, solutions of trypsin or chemotripsin. Of particular importance is the use of soft hydrophilic contact lenses, sealed glasses to reduce the evaporation of moisture from the surface of the eyes. The secretory activity of the lacrimal glands can be stimulated by the use of bromhexine in a daily dose of 24-48 mg.

With at least partially preserved tearing, minor surgical interventions are performed to block tear secretion through the nasolacrimal canals. In case of accession of a secondary infection, antimicrobial and antifungal drugs are prescribed.

In patients with xerostomia, regular rinsing of the mouth should be prescribed. To stimulate the secretion of saliva, you can use mint and citrus lollipops that do not contain sugar, pilocarpine tablets. The effectiveness of cevimeline, an acetylcholine derivative with high similarity to muscarinic receptors in the epithelium of the salivary and lacrimal glands, has been proven.

To prevent dental complications, you should carefully observe oral hygiene, often use fluoridated toothpaste, add sodium fluoride to rinse solutions. For the treatment of secondary candidiasis, fluconazole, nystatin are prescribed. In the local treatment of sialadenitis, applications of dimexide with hydrocortisone, heparin are used. Perform 10-15 procedures for 20-30 minutes.

With dryness of the trachea and bronchi, bromhexine, acetylcysteine ​​are prescribed, with dry skin, vagina - emollient creams, gels. A necessary condition for the effective treatment of Sjögren's syndrome is the therapy of the underlying disease.

What diseases can be associated

Organ lesions due to Sjögren's disease affect many organs and systems and are manifested by such diseases:

• salivary glands - parenchymal sialadenitis, enlargement of the salivary glands, hypofunction of the salivary glands;
• visual system and lacrimal glands - dry conjunctivitis, blepharoconjunctivitis, dry keratoconjunctivitis, deep corneal xerosis, hypolacrimia;
• mucous membranes of the oral cavity, nasopharynx, trachea, bronchi, vagina - stomatitis, dry rhinopharyngolaryngitis, dry tracheobronchitis, dry colpitis;
• reticuloendothelial system - regional or generalized lymphadenopathy, hepatomegaly, pseudolymphoma, lymphoma;
• joints and muscles - arthralgia, recurrent non-erosive arthritis, myositis, myalgia;
• cardiovascular system - Raynaud's syndrome, vasculitis, purpura;
• lungs - interstitial pneumonia, alveolar pulmonary fibrosis, recurrent pneumonia;
• kidneys - tubular acidosis, immunocomplex glomerulonephritis, diffuse glomerulonephritis;
• digestive tract - hypotension of the esophagus, atrophic gastritis, pancreatitis;
• nervous system - neuritis of the trigeminal and facial nerves, polyneuropathy, polyneuritis, cerebrovasculitis.

Due to the development of atrophic tracheobronchitis, recurrent bacterial pneumonia and bronchitis are often complications. In patients with Sjögren's disease, the risk of developing beta-cell lymphomas is increased by 44 times compared with the general population. The most prone to the development of malignant lymphomas are patients with lymphadenopathy, significant and prolonged enlargement of the parotid glands, their irradiation, skin ulcerative necrotic vasculitis, peripheral neuropathy, persistent low fever, anemia, lymphopenia and hypocomplementemia. The development of lymphoma should be suspected in the event of the appearance or persistence of fever and lymphadenopathy against the background of a decrease in the immunological activity of Sjögren's disease (a decrease in the content of beta-globulins, rheumatoid factor titer, etc.).

Treatment of Sjögren's syndrome at home

Treatment of Sjögren's syndrome permissible at home, however, the patient must be registered at the dispensary, periodically visit a medical institution for examinations and medical consultations. A revision of the treatment strategy based on their results is likely.

The prognosis for life in Sjögren's disease is generally favorable. It worsens significantly with the development of lymphomas. Early adequate therapy with the use of glucocorticoids and cytostatics several times reduces the risk of developing lymphomas, improves the course of the disease and labor prognosis. Timely treatment can prevent severe ophthalmic, dental and systemic complications.

Prevention is aimed at preventing exacerbation, progression of Sjögren's disease and the development of complications (infections, lymphomas). This is achieved by early diagnosis and adequate treatment, the content of which varies depending on the activity, course and stage of the disease. It is important to purposefully search for signs of Sjögren's syndrome in rheumatoid arthritis, systemic connective tissue diseases, and liver diseases.

What drugs to treat Sjögren's syndrome?

In the initial stage, aminoquinoline drugs are prescribed:

• - 0.4 g per day; usually combined with 5-10 mg;
• - 0.25 g per day; usually combined with 5-10 mg of prednisolone.

For systemic lesions:

• - 4-8 mg in combination with glucocorticosteroids;
• - 200 mg intramuscularly 1-3 times a week.

Persons with a crisis course of Sjögren's disease with the development of generalized vasculitis or with massive lymphoplasmacytic infiltration of organs and tissues:

• - 1000 mg per 1 m 2 of body surface intravenously in 200 ml of isotonic sodium chloride solution or 5% glucose solution for 40-60 minutes for 3 consecutive days;
• - 1000 mg per 1 m 2 of body surface is combined with methylprednisolone on the 2nd day.

Treatment of Sjögren's syndrome with folk methods

Treatment of Sjögren's syndrome does not involve the use of folk remedies, since they do not have a mechanism for influencing changes in exogenous glands and connective tissue that occur during the disease.

Treatment of Sjögren's syndrome during pregnancy

Sjogren's disease is not a contraindication to pregnancy, but only in the absence of functional disorders of the internal organs. Usually, the management of pregnancy and childbirth is determined by the activity of the disease and the risk of its combination with scleroderma, systemic lupus erythematosus, and arthritis. Pregnancy usually does not affect the course of the disease, if before that it was taken under control.

A woman with Sjögren's syndrome is shown hospitalization in the early stages to clarify the diagnosis, identify the features of the course of the disease and resolve the issue of the possibility of continuing the pregnancy and developing treatment tactics.

Which doctors to contact if you have Sjögren's syndrome

Typical changes in Sjögren's disease are an increase in ESR, CRP levels, hyper- and dysproteinemia, polyclonal hyper-beta-globulinemia, in some patients - moderate anemia and leukopenia. In more than 80% of cases, RF and ANF of a mottled type of luminescence in high titers are detected. At the same time, antibodies to DNA are found very rarely.

The most important immunological markers of Sjögren's disease (specificity reaches 90%) are antibodies to soluble nuclear antigens Ro (SS-A) and La (SS-B), they are found in 70% of patients. In Sjögren's syndrome, a positive test for these antibodies occurs 2-4 times less often, and against the background of RA and SJS, antibodies to Ro (SS-A) are found much more often than in La (SS-B). It is important that high titers of antibodies simultaneously to both antigens are found only in Sjogren's disease and SLE with concomitant Sjogren's syndrome, and a positive test for antibodies to La (SS-B) in the absence of antibodies to Ro (SS-A) is found only in Sjogren's disease.

Characteristic of Sjögren's disease is an increase in the content of several or all classes of immunoglobulins, a high level of the CEC. In a third of patients, cryoglobulins are found in the blood, in some - with concomitant hypocomplementemia.

To objectify xerophthalmia, Schirmer's test is used: coloring of the cornea and conjunctiva with Bengal pink. The Schirmer test is performed as follows: the bent end of a strip of laboratory filter paper 5-6 mm wide is inserted behind the lower eyelid, the patient is asked to close his eyes and after 5 minutes the length of the moistened strip is estimated, starting from the bend. The test is considered positive if the length of the moistened segment is less than 15 mm. Bengal rose (1% solution) is applied to the conjunctiva and cornea and the presence of the dye and the localization of its fixation are assessed. Areas where there is no epithelium or it is changed are painted over. This indicates severe dryness of the eyes, which has led to damage to the corneal tissue. With xerophthalmia, the zone of maximum fixation of the dye is located parallel to the palpebral fissure, where contact with air and moisture evaporation are long.

The defeat of the minor salivary glands can be proven by examining a biopsy of the mucous membrane of the lower lip. There is no need for a biopsy of the large glands, since the processes of infiltration in the small and large salivary glands proceed in parallel. Histological signs of lesions of the salivary glands in Sjogren's disease and Sjogren's syndrome are the same.

For the diagnosis of parenchymal parotitis, sialography is also used (X-ray examination of the parotid glands after the introduction of a radiopaque substance into the parotid strait), sialometry (assessment of unstimulated and stimulated saliva flow), scintigraphy (assessment of the rate of saliva secretion).

Clinical and laboratory manifestations depend on the variant of the course and the degree of disease activity.

Chronic course is noted in 40% of patients, more often in the elderly. The disease begins gradually, imperceptibly for the patient. Signs of damage to the exocrine glands, primarily salivary and lacrimal glands, predominate, systemic manifestations are almost absent. The first manifestations are a gradual increase in the parotid salivary glands, dryness, itching and burning in the eyes, dry mouth, progressive cervical caries, arthralgia. Laboratory parameters changed a little, with exacerbations, a moderate increase in ESR, a slight hyper-beta-globulinemia (up to 26%) are observed, and rheumatoid factor is found in low titers.

Subacute course is typical for most patients, when the disease debuts at a young age. The onset of the disease is quite bright: parotitis develops with local signs of inflammation, fever, polyarthritis or intense arthralgia, often in combination with conjunctivitis, hemorrhagic rash. The analyzes reveal anemia, a significant increase in ESR, the content of beta-globulins, CEC, high titers of RF and ANF. High laboratory activity persists throughout the disease.

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

Sjögren's syndrome - what is this disease?

The Swedish ophthalmologist Sjogren first drew attention to the complex of symptoms of dry syndrome 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. Observation interested other doctors and scientists. It turned out that this pathology is common and specific treatment is required to combat it.

Sjögren's syndrome - what is it? This chronic autoimmune disease appears against the background of malfunctions of the immune system. The body takes some of its own cells as 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 external secretion glands - usually salivary and lacrimal.

Sjögren's syndrome - causes

To say unequivocally why autoimmune diseases develop, medicine cannot yet. Therefore, where dry Sjögren'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, the impetus for the development of the disease is viruses - cytomegalovirus, Epstein-Barr, herpes - or diseases such as polymyositis, systemic scleroderma, lupus erythematosus, rheumatoid arthritis.

Primary Sjögren's syndrome

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

Secondary Sjögren'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 connective tissue lesions.

Sjögren's syndrome - symptoms

All manifestations of the disease are usually divided into glandular and extra-glandular. The fact that Sjögren's syndrome has spread to the lacrimal glands can be understood by the sensation of burning, "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 occurs, the palpebral fissures noticeably narrow, visual acuity deteriorates. Enlarged lacrimal glands are rare.

Characteristic signs of Sjögren's syndrome, which affected the salivary glands: dryness of the mucous membrane in the mouth, red border, lips. Often, seizures form in patients, and, in addition to salivary glands, some nearby glands also increase. At first, the disease manifests itself only during physical exertion or emotional overstrain. But later, dryness becomes permanent, the lips become covered with crusts that crack, which increases the risk of a fungal infection.

Sometimes, due to dryness in the nasopharynx, crusts begin 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 become 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.

Extra-glandular manifestations of the symptom complex of Sjögren's syndrome look like this:

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

Sjögren's syndrome - differential diagnosis

The definition of the disease is mainly based on the presence of xerophthalmia or xerostomia. The latter is diagnosed using sialography, parotid scintigraphy and salivary gland biopsy. For the diagnosis of xerophthalmia, the Schirmer test is performed. One end of a strip of filter paper is placed under the lower eyelid and left 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, percynos anemia, drug-induced disease. The identification of primary dry syndrome is significantly facilitated by the detection of SS-B antibodies. It is most difficult to diagnose Sjögren's disease with rheumatoid arthritis, because joint damage begins long before signs of dryness appear.

Sjögren's syndrome - tests

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

1. In the general blood test, an 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 revealed 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. The results of a salivary gland biopsy confirm the symptoms of Sjögren's syndrome.

Sjögren's syndrome - treatment

This is a serious problem, but it is not fatal. If you pay attention to its signs in time and start treatment with a diagnosis of Sjögren'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 strengthen the immune system, will not allow the pathological process to actively develop and significantly reduce the risk of complications.

Can Sjögren's syndrome be cured?

Once the diagnosis is confirmed, the patient receives therapeutic advice. Sjögren's syndrome is 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 appointments help, laboratory parameters and histological picture improve.

Dry syndrome therapy involves alleviating symptoms and, if necessary, combating an underlying autoimmune disease. Before treating Sjögren's syndrome, a diagnosis is necessarily carried out. After that, as a rule, such means are used:

• glucocorticoid Prednisolone (the regimen 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;
• Kontrykal or Trasilol - drugs that stop the production of proteolytic enzymes;
• Cyostatics - Chlorbutin, Azathioprine, Cyclophosphamide (mostly recommended to be taken together with glucocorticoids).

To get rid of dry mouth, rinsing is prescribed. Dry eye syndrome is treated by instillation of saline solution, Hemodez. Bromhexine can help dry bronchi and trachea. Applications with Dimexide, Hydrocortisone or Heparin fight inflammation of the glands. Sometimes dry mouth with a diagnosis of Sjögren's syndrome leads to the development of dental diseases. To prevent them, you need to take care of maximum oral hygiene.

Sjögren's disease - treatment with folk remedies

Dry syndrome is a complex of symptoms and signs. With all of them it is better to fight traditionally. But sometimes, with Sjogren's syndrome, alternative methods used in parallel help to improve the patient's condition. Some patients, for example, note that dill and potato juice eye drops are much more effective than pharmacy lacrimal fluids.

Herbal decoction for rinsing

Ingredients:

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

Preparation and application:

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

Sjögren's syndrome - prognosis

This disease proceeds without threat to life. But because of it, the quality of life of patients is noticeably deteriorating. Treatment helps prevent complications and preserves the ability of adults to work - Sjögren'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.