Sjögren's disease (syndrome): what it is, causes, symptoms and treatment. Sjögren's disease (sicca syndrome): what is it, causes, symptoms and treatment What kind of Sjögren's disease is it?
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Sjögren's disease or Sjögren's syndrome is a complex of several diseases that complement each other. Patients complain of a feeling of dryness in the eyes and mouth, as well as pain in the joints. This pathology is quite common among the adult population; postmenopausal women and elderly people are especially susceptible to Sjögren's syndrome due to disruption of the lacrimal glands. From this article you can learn in detail about Sjögren's disease - what it is and which doctors you should contact.
Description of the disease
The main characteristics of the disease are the following conditions:
Usually, to establish the presence of the disease, signs of dysfunction of the exocrine glands - Bartholin's, sebaceous, and sweat glands - are sufficient. In about a third of cases, the muscular system, vascular system, kidneys, respiratory organs, gastrointestinal tract, and musculoskeletal system are affected.
The disease is characterized by a slow increase in symptoms and the involvement of more and more organs and systems in the pathological process. Systemic, dental and ophthalmological failures lead to complete loss of ability to work. Patients with Sjögren's disease are at high risk of developing malignancies in the lymphatic system.
Causes of Sjögren's syndrome
The mechanism of development of the disease is autoimmune in nature. The result of exposure to various provoking factors is a dysfunction of the immune system. As a result of this, the defense system perceives the body’s own organs and tissues as foreign and actively destroys them. This phenomenon may be caused by the following factors:
- hereditary predisposition;
- development of infectious diseases;
- constant stress reactions of the body in response to external stimuli;
- the presence of chronic fatigue syndrome;
- overdose or abuse of medications (so-called “chemical stress”);
- regular hypothermia or overheating of the body;
- a tendency to such types of allergic reactions as anaphylactic shock, Quincke's edema.
Symptoms
Very often, patients complain of discomfort arising from dryness of the oral mucosa, while an increase in the parotid or submandibular salivary glands is observed (we recommend reading: how and with what can enlarged salivary glands be treated?). Pinpoint hemorrhages appear in the eyes, photophobia and pain occur. Symptoms of Sjogren's disease are divided into two main groups:
- glandular - pathological changes and dysfunction of the epithelial glands occur;
- extraglandular signs of the disease vary significantly due to the individual course of the disease of each patient.
Feeling of dry mouth (xerostomia)
Patients are concerned about dry mouth, which indicates the onset of the disease. Often the development of pathology is accompanied by low fever and skin rash. The salivary glands are affected first, and there is a decrease in saliva production. One of the main signs of Sjögren's syndrome is the appearance of an unusual sour taste in the mouth. The mucous membrane, having lost the protective properties of saliva, dries out. The development of stomatitis, candidiasis, and the appearance of multiple caries is possible.
Difficulty swallowing
A small amount of saliva or its complete absence leads to dry lips and the appearance of congestion in the corners of the mouth. At the initial stages of the development of the disease, dry mouth appears only with increased physical activity or severe anxiety; over time, the situation worsens (we recommend reading: dry mouth at night: causes and symptoms of what disease could this be?). There are difficulties when swallowing, the oral mucosa becomes bright red. As Sjögren's syndrome develops, signs of damage to the gastrointestinal tract appear. The process of eating food becomes painful and unpleasant. The consequences of the pathology are the development of chronic diseases of the gastrointestinal tract - pancreatitis, colitis.
Lack of saliva (cheilitis)
With a constant lack of saliva, a thick coating appears on the tongue, bad breath may occur, and the surface of the lips becomes covered with dry crusts. Drying of the nasopharynx provokes the development of concomitant diseases, for example, otitis media. Patients often notice a change in their voice, which becomes hoarse or hoarse. An insufficient amount of saliva leads to a decrease in the secretion of gastric juice and pancreatic enzymes involved in the digestion of food.
Death of tongue papillae
With Sjögren's disease or syndrome, pathological changes also affect the tongue. Its surface becomes loose, discomfort and a burning sensation appear. Often patients cease to distinguish the taste of food. This occurs due to flattening or complete atrophy of the taste buds of the tongue. The tongue takes on an unusually bright color, and there is a feeling of numbness on the surface, including the lateral areas. A “folded” tongue and other signs of the disease can be seen in the photo accompanying the article.
Enlarged parotid glands (mumps)
In Sjögren's disease, an inflammatory process develops in the parotid glands. The salivary glands increase significantly in size, swelling appears, and purulent secretion occurs through the salivary ducts. Patients' body temperature rises to 39-40°C, and difficulty opening their mouth occurs. Often patients try to insulate the parotid area in order to get rid of discomfort.
Lack of tear fluid (xerophthalmia)
Impaired tear production is considered one of the characteristic signs of Sjögren's disease. In the case of a combination of the underlying disease with rheumatoid arthritis, the lack of tear fluid is more pronounced. Symptoms of the disease are the following sensations:
![](https://i1.wp.com/pro-zuby.com/wp-content/uploads/image001-300x181.jpg)
The upper and lower eyelids swell and itch. The appearance of eye fatigue and pain with slight strain is noted. In some cases, a significant decrease in visual acuity and the development of photophobia are possible.
Joint discomfort
Sjögren's disease, among other symptoms, is characterized by discomfort in the joints, a feeling of stiffness and muscle pain, which is especially noticeable after waking up. Basically, the pathological process affects the knee and elbow joints, as well as the wrists and fingers. At the same time, there is a slowdown in the growth of nails, which become brittle and thin.
Other signs
Pathological changes occur in the mucous membrane of the external genitalia in women. Patients report a burning sensation, dryness, and itching at the vaginal opening. During sexual intercourse, discomfort and pain occur due to insufficient production of natural lubrication.
In the initial stages of the disease, sweating decreases and severe dryness of the skin appears due to damage to the glands responsible for the production of sebum. Over time, ulcers and open wounds form on the skin, and hives may appear.
The pathological process involves the kidneys and digestive organs. The disease can provoke the development of cirrhosis of the liver, in which the tissue of the organ is destroyed. Patients complain of a feeling of bitterness in the mouth and indigestion. The lining of the stomach also dries out, preventing normal digestion. The consequence of this is the formation of ulcers, and acute pancreatitis is often diagnosed.
In some cases, the formation of malignant neoplasms is observed at the site of the salivary glands. If measures are not taken to detect and timely treat the disease, the immune system fails completely - skin cancer occurs.
How is the disease diagnosed?
When diagnosing the disease, it is necessary to exclude a number of pathologies that have similar symptoms, for example, diabetes mellitus or Parkinson's disease. General dehydration and the use of anticholinergic drugs can also cause dry mucous membranes.
Due to the generalization of the process, it is necessary to conduct a comprehensive examination of all systems and organs. To prescribe effective treatment, a neurologist, orthopedist, rheumatologist, and dentist are involved in making a diagnosis. Consultation with a gastroenterologist and ophthalmologist is mandatory.
To take into account all possible complications, laboratory and instrumental tests are prescribed:
- determination of antinuclear antibodies;
- Shimer's test;
- sialometry;
- tissue biopsy of the salivary glands;
- Ultrasound or MRI;
- eye examination using a special lamp.
Treatment methods
When treating Sjögren's syndrome, the main specialist is a rheumatologist. The disease is susceptible to the influence of hormonal and cytostatic drugs. For systemic lesions, such as glomerulonephritis, polyneuritis, ulcerative necrotizing vasculitis, plasmapheresis is prescribed. To eliminate the consequences of xerophthalmia, it is recommended to use artificial tears, as well as washing the eyes with antiseptic solutions. For inflammatory processes of the parotid glands, applications with medications are used. Artificial saliva is used to relieve dry mouth. A good result in restoring the oral mucosa is achieved by using sea buckthorn and rosehip oils as applications. To prevent the development of caries, it is necessary to see a dentist.
During the treatment of Sjögren's disease, special attention is paid to restoring the function of the gastrointestinal tract. For secretory insufficiency, long-term replacement therapy using natural gastric juice, hydrochloric acid and enzymes is recommended.
Sjögren's disease or syndrome most often occurs in people with rheumatoid diseases. Age factors and gender are also directly related to the development of the disease. These categories of patients need to more carefully monitor their health. Timely initiation of treatment makes it possible to avoid complications that significantly reduce the quality of life and often lead to disability. The course of the disease can be controlled over a long period of time, with alternating periods of exacerbation and remission. It cannot be completely cured and remains with patients for the rest of their lives.
The prognosis with correctly prescribed therapy is favorable - it is possible to slow down the development of the disease and restore ability to work. For the purpose of prevention, it is necessary to avoid excessive stress on the visual organs, stressful situations, and strictly follow all the instructions of the attending physician.
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Sjögren's syndrome ("siggy syndrome") is an autoimmune lesion of the exocrine (primarily lacrimal and salivary) glands, accompanied by their hypofunction and usually combined with systemic immuno-inflammatory diseases. This definition, which seems to us the most accurate from a scientific point of view, quite fully characterizes the essence of this pathology.
The diagnostic triad of K. Bloch et al. is popular among clinicians. (1965):
1) dry keratoconjunctivitis with enlarged or normal size of the lacrimal glands;
2) xerostomia with enlarged or normal size of the salivary glands; 3) the presence of a systemic connective tissue disease (more often rheumatoid arthritis (RA), less often systemic lupus erythematosus (SCR), systemic scleroderma (SSD), even less often - polyarteritis nodosa or dermatomyositis).
Most authors consider two of these three manifestations sufficient and necessary for the diagnosis of Sjögren's syndrome. The combination of sicca with RA or another connective tissue disease is considered secondary Sjögren's syndrome, and the presence of only keratoconjunctivitis sicca and xerostomia is considered primary Sjögren's syndrome (or "Sjögren's disease", in less accepted terminology). The disease occurs in representatives of all races and at different ages (including children). More than 90% of all patients are middle-aged and elderly women.
Etiology and pathogenesis
The etiology and pathogenesis are not clear. It is known that T and B lymphocytes are present in 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 histocompatibility antigens DR3 and B8. Indirect evidence of the role of heredity and autoimmune disorders in Sjögren's syndrome is the frequent manifestations of sicca syndrome in pure NZB/W and MRL mice with clear signs of autoimmune pathology.Main histomorphological feature- lymphocytic and plasma cell infiltration of salivary, lacrimal and other exocrine glands - bronchial, gastrointestinal, vaginal. Typically, damage occurs to both large salivary glands (parotid, submandibular) and small ones located in the mucous membrane of the gums and palate. At the first stages, the infiltrate is located around small intralobular ducts; later it spreads throughout the parenchyma of the gland, sometimes accompanied by the formation of germinal lymphoid centers and atrophy of glandular tissue with its replacement by adipose tissue.
Characterized by proliferation and metaplasia of the cells lining the ducts, with the development of so-called myoepithelial islands, which are found in biopsy specimens of the salivary glands in 30-40% of cases. The general lobular structure of these glands is usually preserved; in a number of patients some lobules are unchanged, others are almost completely destroyed. The size of the affected glands can be either enlarged or normal. It is important to note that, although clinical manifestations of Sjogren's syndrome are observed in a clear minority of patients with diffuse connective tissue diseases, subclinical histological signs of inflammation of the salivary glands are found in almost 100%. In particular, J. Waterhouse and J. Doniach (1966) found lymphocytic infiltrates in the submandibular glands in all those who died with rheumatoid arthritis.
Lymphoid infiltrates can in some cases occur not only in the exocrine glands, but also in the lungs, kidneys and skeletal muscles, sometimes leading to corresponding functional disorders. In some patients, lymphoid infiltrates in the salivary glands, lymph nodes and internal organs lose their usual inflammatory benign character.
The cells acquire a “younger” appearance and polymorphism, and show signs of invasive spread (in particular, the structure of the lymph nodes can be completely erased). In such cases, it is not always possible to clearly distinguish morphologically between benign and malignant lymphoproliferation, which is why the term “pseudolymphoma” arose. In some patients, the histological picture corresponds to immunoblastic lymphadenopathy; the development of true lymphosarcoma is also possible.
Clinical picture
Xerophthalmia, i.e. dry eye itself, associated with insufficient tear production, 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 dilation of the conjunctival and pericorneal vessels.A small amount of crumbly or thick viscous white or yellowish discharge is typical. In patients, tear production is reduced, up to the complete absence of tears, which is especially noticeable with severe negative emotions (inability to cry) and exposure to irritating substances (in particular, lack of tear production when peeling onions). As a rule, patients feel better in areas with a humid and cool climate.
Objective diagnosis of dry keratoconjunctivitis is possible by staining the altered areas of the conjunctiva and cornea with a 1% solution of rose bengal (the results of this technique, however, are not always interpreted unambiguously) and especially using a slit lamp, which makes it possible to reliably establish punctate and filamentous keratitis. To recognize decreased tear production, the most popular is the Schirmer test, which consists of the following.
A strip of filter paper 5 mm wide is placed with its upper end behind the lower eyelid at the border of its middle and inner third. Normally, paper gets wet by at least 15 mm in 5 minutes. With lower results, tear secretion can be stimulated by inhaling a 10% ammonium chloride solution, the bottle of which is placed 15 cm below the nose (“forced Schirmer test”). A positive Schirmer test (i.e. the very fact of insufficient tear production) is not synonymous with keratoconjunctivitis sicca, especially if this test is carried out at high temperatures in a dry room.
Clinically noticeable enlargement of the lacrimal glands in Sjogren's syndrome occurs in no more than 5% of patients. Dry eyes in this disease cannot be associated only with the destruction of the parenchyma of the lacrimal glands by the inflammatory infiltrate, since surgical removal of these glands (for other reasons) does not lead to such pronounced dryness. Apparently, the lesion of all mucous glands of the conjunctival sac in Sjögren's syndrome is of decisive importance in the development of xerophthalmia. Perhaps a change in the biochemical composition of tears may play a certain role: a decrease in viscosity and an increase in water content makes it impossible to create a uniform layer of tears over the surface of the cornea.
Untreated dry keratoconjunctivitis can be complicated by secondary infection, which is primarily due to the weakening or absence of the bactericidal effect of lysozyme contained in the normal secretion of the lacrimal glands. As a result of infection (bacterial or viral), multiple complications are possible - fusion of the eyelids with the eyeball, loss of the blink reflex, ulcerative keratitis, perforation of the cornea with the threat of uveitis, secondary glaucoma and loss of vision, which is very rare.
Xerostomia, if it exists in isolation, should not be considered as a mandatory manifestation of Sjögren's syndrome. Dry mouth occurs in older people, alcoholics, smokers, and people who breathe through their mouths or take antidepressants. Temporary severe dry mouth also occurs with nervous excitement. In addition, patients with Sjögren's syndrome may experience only very modest sensations of dry mouth and therefore do not actively complain about it. Moreover, even with indisputable histological signs of the disease, the amount of saliva sometimes remains sufficient for the patient not to experience discomfort.
Severe xerostomia in Sjögren's syndrome can be very distressing. The greatest difficulties arise when chewing and swallowing solid food, and therefore patients are forced to constantly drink water. Attempts to combat dryness by sucking lemon or sour candies are successful only in the early stages. In severe cases, food particles “stick” to the gums, cheeks and palate, and patients are forced to remove them with their hands. Dryness of the pharyngeal mucosa may cause dysphagia; in some cases, a true violation of the mobility of the esophagus was noted.
Painful cracks in the lips and corners of the mouth are very common. The mucous membrane of the mouth is often irritated, its surface tissue easily peels off, and eating hot or spicy food causes pain. When drinking milk, its coagulated threads that linger on the mucous membrane of the cheeks are sometimes incorrectly interpreted as fungal lesions (which in some cases can actually complicate xerostomia in Sjögren's syndrome). Painful atrophic glossitis with cracked tongue is not uncommon. Ulcerative stomatitis is also possible. Normal accumulation of saliva at the base of the frenulum of the tongue is not observed in clinically severe cases. It is also not possible to increase salivation after massage of the parotid and submandibular glands.
In most patients, enlargement of the parotid glands is unilateral and temporary, although it is possible to maintain normal size or to have bilateral enlargement. The submandibular glands are less frequently enlarged. Enlarged salivary glands are often slightly painful on palpation and are usually dense, with a smooth or uneven surface. At the same time, they do not have the stony density or nodularity characteristic of tumors. Acute pain in the glands, combined with fever, local hyperemia and hyperthermia, most often indicates the addition of a secondary infection, which is usually a consequence of the often observed dilation and tortuosity of the intraglandular salivary ducts.
To judge the pathology of these ducts, as well as the degree of atrophy of the gland parenchyma, contrast sialography can be successfully used. The severity of destruction of the glandular parenchyma is also determined by scintigraphy using labeled technetium (99m Te). In this case, the level of absorption of the radionuclide by the tissues of the salivary glands and, as a control, the thyroid gland is compared.
It is peculiar that the amount of saliva secreted per unit of time, which, it would seem, should characterize xerostomia especially accurately, is in fact not a sufficiently reliable indicator, since it is subject to sharp individual fluctuations. For a general judgment about a decrease in salivation, it is sometimes important to verify the small stimulating effect of acidic foods, such as lemon.
With an insufficient amount of saliva, gingivitis, bad breath, caries with toothache are common. Teeth crumble easily and previously placed fillings fall out. It should be borne in mind that drug-induced xerostomia is almost never the cause of dental pathology.
Changes in the mucous membranes of the external genitalia
The apocrine glands of the female external genitalia are often affected in Sjogren'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 a consequence of estrogen deficiency rather than dry syndrome as such.Systemic manifestations of Sjogren's syndrome often present serious difficulties in their pathogenetic interpretation.
They can: 1) be symptoms of specific connective tissue diseases, with which Sjögren’s syndrome is often combined; 2) reflect the actual pathogenetic features of the syndrome in question and 3) be a nonspecific consequence of insufficient function of the exocrine glands with dry mucous membranes, etc. Obviously, the first category of symptoms does not relate to Sjögren’s syndrome as such and is not considered in this section.
Skin changes
A common symptom of Sjögren's syndrome- dry skin, although patients relatively rarely actively complain about it. In most patients, increased ambient temperature, physical activity, or injections of pilocarpine cause noticeable sweating. However, in some cases this does not happen and skin biopsy reveals inflammatory changes in the area of the sweat glands with destruction of their structure. Skin. changes that reflect vasculitis (ulcers, hemorrhages) are discussed below.Very often observed hives, which is a reflection of drug allergies characteristic of Sjögren's syndrome. K. Whaley et al. (1973) even found that allergic reactions to penicillin in RA almost exclusively occur in patients with concomitant Sjogren's syndrome.
Damage to joints and muscles
From 50 to 70% of all patients with Sjogren's syndrome have a concomitant inflammatory connective tissue disease (usually rheumatoid arthritis), which determines the nature of the joint pathology. However, among patients with primary Sjogren'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 possible recurrence, erosion does not occur. Reverse development of inflammatory changes occurs without treatment.The most commonly affected joints are the knee and elbow joints. The pathogenesis of this variant of arthritis is unknown. There is an opinion about its immunocomplex nature. An indirect argument in favor of this point of view is the frequent combination of Sjogren's syndrome and RA, each of which is characterized by a high level of circulating immune complexes and the virtual absence of dry syndrome in patients with psoriatic arthritis and other seronegative spondyloarthritis, for which the formation of immune complexes is completely uncharacteristic.
It should be borne in mind that although in most patients with a combination of RA and Sjögren's syndrome, signs of the rheumatoid process long precede sicca syndrome, the opposite relationship is also possible. Therefore, at the first appearance of articular joints in a patient with long-term isolated Sjögren's syndrome, the onset of true rheumatoid arthritis cannot be ruled out.
Severe muscle damage in primary Sjögren's syndrome almost never occurs; there are only a few corresponding descriptions. Patients in some cases complain of mild myalgia and moderate muscle weakness, and the latter may also be associated with electrolyte disturbances and tubular acidosis. Muscle biopsy in patients with primary Sjogren's syndrome often reveals clinically asymptomatic focal myositis with perivascular lymphoid infiltration, as well as deposition of immunoglobulins and complement in muscle tissue and dystrophic changes in myofibrils under electron microscopy.
Respiratory system
Almost 50% of patients with Sjögren's syndrome report dry nasal mucous membranes. In some cases, it is significant and is accompanied by the formation of painful hard crusts and nosebleeds. Taste and smell sensations may be weakened or changed. Upon examination, atrophy of the nasal mucosa is found in many patients. Sometimes there is dryness of the vocal cords, which may be coated with viscous mucus. These changes lead to hoarseness of the voice. A sharp decrease in hearing and serous otitis media are possible due to the closure of the nasopharyngeal opening of the auditory tube by dry crusts; Removing the crusts can lead to rapid improvement.Often the so-called dry bronchitis, in which thick mucous discharge causes a constant dry cough with difficulty in expectoration and the addition of a secondary infection. In the development of the latter, a certain importance is also attached to insufficient local IgA production.
Infiltrates of the walls of the bronchioles and alveolar septa by T and B lymphocytes are considered relatively specific for Sjögren's syndrome, which can clinically manifest as symptoms of airway obstruction with cough and shortness of breath, as well as episodes of usually mild interstitial pneumonia. Mild short-term pleurisy that resolves spontaneously within 1-2 weeks has often been described. Chronic interstitial inflammatory process (fibrosing alveolitis) is relatively rare.
The cardiovascular system in Sjögren's syndrome does not undergo any specific pathological changes primarily associated with this disease.
Kidney pathology
A number of patients with Sjogren's syndrome showed hidden or obvious signs of impaired renal tubular function, manifested by tubular acidosis, aminoaciduria and renal glycosuria. The mechanisms of development of these disorders have not been fully elucidated. There is, in particular, an assumption that they are associated with chronic pyelonephritis or with increased blood viscosity as a result of hyperglobulinemia, often characteristic of Sjogren's syndrome. It is more likely that this pathology is directly related to immune reactions, since infiltration of the medullary layer of the kidneys with lymphocytes and plasma cells is found in the corresponding patients.This view is supported by the fact that tubular acidosis in Sjögren's syndrome improves after treatment with cytostatic immunosuppressants. In addition, it is noteworthy that during an autoimmune crisis of rejection of a transplanted kidney, tubular acidosis also develops in combination with peritubular lymphoid infiltration. Since the involvement of the tubules in the process can manifest itself in a decrease in the response to antidiuretic hormone, when taking an increased amount of fluid (due to xerostomia), it sometimes leads to the development of a symptom complex of diabetes insipidus.
In rare cases, patients with Sjogren's syndrome develop acute glomerulonephritis, combined with hypocomplementemia and deposition of immunoglobulins and complement in the main membrane of the glomeruli. It is important to note that this is not about concomitant SLE, in which the development of Sjögren's syndrome is quite possible, since none of these patients had antibodies to native DNA, which are highly specific for patients with lupus nephritis.
Digestive and abdominal organs
Dryness of the esophageal mucosa, which occurs with severe Sjögren's syndrome, can cause mild dysphagia when eating solid foods in some patients. Chronic atrophic gastritis with markedly reduced acidity is not uncommon, sometimes combined with the presence of antibodies to parietal cells. Acute pancreatitis has been described in some patients. No serious intestinal pathology or intestinal absorption disorders were observed.A slight enlargement of the liver and spleen in combination with moderate laboratory signs of liver dysfunction is not so rare. Relatively often, antibodies to mitochondria, smooth muscle and membranes of liver cells are detected, which is considered a definite indication of the participation of the liver in the immune pathological process. The possibility of the development of a specific liver pathology (chronic active hepatitis or primary biliary cirrhosis) as a manifestation of Sjögren's syndrome (as some authors point out) does not seem to be proven sufficiently convincingly.
In such cases it cannot be ruled out reverse cause-and-effect relationships, since in these autoimmune diseases secondary Sjögren's syndrome often occurs (just as in RA in combination with dry syndrome, joint damage cannot be considered a manifestation of Sjögren's syndrome). Among the possible endocrine pathologies, one should point out histological signs of chronic thyroiditis of the Hashimoto type in approximately 5% of patients with Sjögren's syndrome. The autoimmune nature of both syndromes makes this combination quite understandable. At the same time, clinical signs of hypothyroidism are very rare.
A number of patients with severe Sjögren's syndrome experience vasculitis of medium and small vessels, manifested clinically by skin ulcers and peripheral neuropathy. It is characterized by a particularly frequent combination with the presence of Ro antibodies. In a number of patients, vasculitis is combined with hyperglobulinemia and is possibly a reflection of immune complex pathology (at least in some cases). Its main symptom is “hyperglobulinemic purpura,” manifested by foci of hemorrhages on the legs.
Elements of hemorrhages in typical cases, they are accompanied by itching and protrude above the surface of the skin, the temperature of which is usually slightly elevated. In rare cases, the rash appears confluent. Hyperglobulinemic purpura is very characteristic of the “dry syndrome”. It is believed that among patients with a combination of hyperglobulinemia and hemorrhages on the legs, 20-30% have or will develop typical manifestations of Sjogren's syndrome. Even with primary sicca syndrome, Raynaud's syndrome occurs more often than in the general population. However, its connection with vasculitis itself seems doubtful.
Changes in the central nervous system and cranial nerves are explained by most authors vasculitis corresponding localization. A wide range of cerebral symptoms has been described, including nystagmus, recurrent hemiparesis and, in particularly severe cases, serous meningoencephalitis. Among the cranial nerves, the trigeminal nerve is relatively more often affected with the development of a characteristic pain syndrome. In isolated patients with Sjogren's syndrome, severe necrotizing arteritis was observed with clinical manifestations corresponding to classic polyarteritis nodosa. However, it cannot be ruled out that polyarteritis in such cases was not a manifestation of Sjögren’s syndrome, but a “concomitant” disease (like RA, etc.).
Sigidin Ya.A., Guseva N.G., Ivanova M.M.
Sjögren's syndrome is an autoimmune disease that causes systemic damage to connective tissues. The exocrine glands - the salivary and lacrimal glands - suffer the most from the pathological process. In most cases, the disease has a chronic progressive course.
Sjögren's syndrome - what is this disease?
The complex of symptoms of dry syndrome was first noticed by the Swedish ophthalmologist Sjögren a little less than a hundred years ago. He discovered that a large number of his patients who came to him with complaints of dry eyes had a couple of other identical symptoms: chronic inflammation of the joints and xerostomia - dryness of the oral mucosa. The observation also interested other doctors and scientists. It turned out that this pathology is common and specific treatment is required to combat it.
Sjögren's syndrome - what is it? This chronic autoimmune disease occurs against a background of malfunctioning of the immune system. The body mistakes its own cells for foreign ones 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 exocrine glands - usually the salivary and lacrimal glands.
Sjögren's syndrome - causes
Medicine cannot yet say unequivocally why autoimmune diseases develop. That’s why it’s a mystery where dry Sjögren’s syndrome comes from. 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 disease. But they appear approximately 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 independently and nothing preceded its appearance, then this is primary Sjögren’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 diagnosed when the disease meets the criteria for problems such 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 extraglandular. The fact that Sjogren's syndrome has spread to the lacrimal glands can be understood by the feeling of burning, “sand” in the eyes. Many people 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 become noticeably narrower, and visual acuity deteriorates. Enlargement of the lacrimal glands is a rare occurrence.
Characteristic signs of Sjogren's syndrome, which affects the salivary glands: dry mucous membranes in the mouth, red border, lips. Often, patients develop jams, and, in addition to the salivary glands, some nearby glands also enlarge. At first, the disease manifests itself only during physical exertion or emotional stress. But later, the dryness becomes permanent, the lips become covered with crusts that crack, which increases the risk of 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 throat and vocal cords become very dry, hoarseness and hoarseness appear. And it also happens that impaired swallowing leads to atrophic gastritis. The diagnosis is manifested by nausea, loss of appetite, heaviness in the epigastric region after eating.
Extraglandular manifestations of the symptom complex Sjogren'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;
- vaginal dryness.
Sjögren's syndrome - differential diagnosis
The definition of the disease mainly relies on the presence of xerophthalmia or xerostomia. The latter is diagnosed using sialography, parotid scintigraphy and salivary gland biopsy. To diagnose 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 Sjögren's syndrome is confirmed, the diagnosis shows that no more than 5 mm is wet.
When making differential diagnoses, it is important to remember that SS can develop in parallel with such diagnoses as autoimmune thyroiditis, pernicious anemia, and drug-induced disease. The determination of primary sicca 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 sicca appear.
Sjögren's syndrome - tests
Diagnosis of the disease involves laboratory tests. When diagnosed with Sjogren's disease, tests show approximately the following results:
- A general blood test reveals an accelerated ESR, anemia and a low level of leukocytes.
- OAM is characterized by the presence of protein.
- Protein is also elevated in the biochemical blood test. In addition, the study reveals the maximum titers of rheumatoid factor.
- A special blood test for the presence of antibodies to thyroglobulin in 35% shows an increase in their concentration.
- 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 when Sjögren’s disease is diagnosed, you can live with it and feel quite comfortable. The main thing for patients is not to forget about the importance of a healthy lifestyle. This will help strengthen the immune system, prevent the pathological process from actively developing and significantly reduce the risk of complications.
Can Sjögren's syndrome be cured?
Once the diagnosis is confirmed, the patient receives therapeutic recommendations. Sjögren's syndrome is currently treated successfully, but it is not yet possible to completely get rid of the disease. 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.
Therapy for sicca syndrome involves alleviating symptoms and, if necessary, combating the underlying autoimmune disease. Before treating Sjögren's syndrome, a diagnosis is required. Afterwards, as a rule, the following means are used:
- glucocorticoid Prednisolone (schedule and dosage are determined individually depending on the severity of the syndrome and the severity of its symptoms);
- angioprotectors - Parmidine, Solcoseryl;
- immunomodulator Splenin;
- anticoagulant Heparin;
- Contrical or Trasylol are drugs that stop the production of proteolytic enzymes;
- Cyostatics - Chlorobutin, Azathioprine, Cyclophosphamide (mainly recommended to be taken together with glucocorticoids).
To get rid of dry mouth, rinses are prescribed. Dry eye syndrome is treated by instilling saline solution, Hemodez. Bromhexine can help dry bronchi and trachea. Applications with Dimexide, Hydrocortisone or Heparin combat inflammation of the glands. Sometimes dry mouth when diagnosed with Sjogren's syndrome leads to the development of dental diseases. To prevent them, you need to take care of maximum oral hygiene.
Sjögren's disease - treatment with folk remedies
Dry syndrome is a whole complex of symptoms and signs. It is better to fight all of them traditionally. But sometimes, in case of Sjögren's syndrome, traditional methods used in parallel help improve the patient's condition. Some patients, for example, note that eye drops made from dill and potato juice are much more effective than pharmaceutical tear fluids.
Herbal decoction for rinsing
Ingredients:
- chamomile flowers - 1 tbsp;
- sage leaves - 1 tbsp. l.;
- water - 1 glass.
Preparation and use:
- Mix the herbs and chop lightly.
- Boil water and pour into the dry mixture.
- The medicine needs to sit for 40 minutes.
- After straining, it is ready for use.
Sjögren's syndrome - prognosis
This disease is not life-threatening. But because of it, the quality of life of patients noticeably worsens. 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, when accompanied by secondary infections such as bronchopneumonia, sinusitis or recurrent tracheitis, sometimes leads to disability.
Sjögren's disease (syndrome) (dry syndrome) is a symptom complex that was first noticed and studied by the Swedish ophthalmologist Sjögren less than 100 years ago. He found that many of his patients who complained of persistent dry eyes also had two other symptoms: dry mouth (xerostomia) and chronic joint inflammation. Subsequently, scientists from other fields of medicine also became interested in this disease. It turned out that this pathology is quite widespread and requires specific treatment.
From this article, the reader will learn what Sjögren's syndrome is, why it occurs, how it manifests itself, and will also become familiar with the principles of diagnosis and treatment of this condition. So…
Sjogren's disease (syndrome) - what is it?
Sjögren's disease (syndrome) is an autoimmune disease in which antibodies to the lacrimal and salivary glands are detected in the blood, disrupting their functions.As mentioned above, Sjögren's disease (syndrome) is a condition characterized by a triad of symptoms: dry eyes (kerato sicca), dry mouth (xerostomia) and some autoimmune disease. Interestingly, even the first 2 signs are enough to make this diagnosis. If a patient is diagnosed with keratoconjunctivitis and xerostomia, Sjögren's syndrome is called primary, and a number of authors consider it a separate nosological entity and call it nothing more than Sjögren's disease. If, along with these signs, chronic inflammation of the joints of an autoimmune nature is determined, Sjögren's syndrome is called secondary.
People of all ages suffer from this pathology, but 90% of those affected are middle-aged and elderly women.
Causes and mechanisms of development of Sjogren's syndrome (disease)
To date, neither the causes nor the mechanisms of occurrence of this pathology have been fully studied. It is believed that the main role in the development of the disease belongs to autoimmune factors. Undoubtedly, there is a genetic predisposition to Sjogren's syndrome: it can be diagnosed simultaneously in several close relatives, most often in a mother and daughter.
Under the influence of unknown factors, a malfunction occurs in the body, as a result of which the lacrimal and salivary glands are infiltrated by B and T lymphocytes. In this case, antibodies to the epithelial cells of the affected glands are detected in the blood, as well as rheumatoid factor and other immunological phenomena.
Pathomorphology
The main morphological feature is the infiltration of the exocrine glands with lymph and plasma cells. First of all, the lacrimal and salivary glands are affected, a little less often – the glands of the bronchi, digestive tract and vagina.
Both large and small glands are affected. At first, at an early stage of the disease, only small ducts are involved in the process; as it progresses, its infiltrate spreads further to the gland tissue itself, as a result of which the glandular tissue atrophies and is replaced by connective tissue. In some cases, the infiltrates described above occur not only in the exocrine (external secretion) glands, but also in other organs and systems of the body, in particular in the muscles, lungs and kidneys. This ultimately leads to dysfunction of the affected organ.
In 30-40% of patients, in the material taken by biopsy of the salivary glands, metaplasia (modification) of the cells lining the ducts is determined: myoepithelial islands appear.
The lobules of the affected glands are destroyed in some patients, while in others the lobular structure is preserved. The glands are either enlarged in size or within normal limits.
Interestingly, even in the absence of clear clinical symptoms of Sjögren's syndrome, a patient with any connective tissue disease will most likely show histological signs of inflammation of the salivary glands.
Symptoms of Sjögren's syndrome
As mentioned at the beginning of the article, the most common signs of this disease are xerostomia, xerophthalmia and chronic inflammation of the joints.
Every patient with Sjögren's syndrome complains of xerostomia, or dryness of the oral mucosa. Dryness at the early stage of the disease occurs periodically, as it progresses it is a constant worry, intensifying with prolonged conversation or against a background of excitement. In addition to direct dryness, the patient notes a burning sensation and pain in the oral cavity, it becomes difficult for him to swallow food - he has to moisten his mouth with liquid before swallowing, but sometimes even this does not help, and the patient cannot swallow dry food. The patient's voice is hoarse, appears suddenly and progresses rapidly - in a short time previously healthy teeth deteriorate and fall out.
During the examination, the doctor pays attention to the reddened, dry mucous membrane of the oral cavity, caries of many teeth, the presence of cracks in the corners of the mouth (in medicine this phenomenon is called cheilitis), as well as the almost complete absence of saliva in the area of the frenulum of the tongue. In severe cases of the disease, the oral mucosa is clearly hyperemic, gingivitis is detected, teeth are absent, the papillae of the tongue are atrophied, the lips are dried out, and areas of peeling are visualized on them. Such a severe course of the disease, as a rule, occurs precisely with Sjögren's disease or with a syndrome that has developed against the background of systemic lupus erythematosus or.
Often, unilateral or bilateral enlargement of the submandibular and/or parotid salivary glands is determined.
Very often, people suffering from Sjogren's syndrome (disease) also suffer from acute inflammation of the parotid glands: mumps. In this case, the gland increases in size, becomes swollen, purulent secretion is released from the salivary ducts, the patient notes an increase in body temperature to febrile levels (39-40 ° C), and he also experiences difficulty opening his mouth.
Dry eyes, or xerophthalmia, is observed in most patients, but it is more pronounced in the case of Sjogren's disease, or when the syndrome is combined with. The patient complains of dryness, cutting pain in the eyes, a feeling of the presence of sand or some foreign body in them. The eyelids are hyperemic and itchy. The eyes get tired quickly, and if they are over-strained, pain appears. Rarely - in especially severe cases - visual acuity decreases and photophobia develops.
In order to recognize reduced tear separation, the Schirmer test is used: a strip of filter paper is placed behind the lower eyelid and monitored when it gets wet. The norm is to get wet by more than 15 mm in 5 minutes. With Sjögren's syndrome, in the same time - 5 minutes - the filter paper gets wet much less. If the result of a simple Schirmer test is low, you can perform a forced test: let the subject inhale a 10% solution of ammonium chloride (ammonia).
The most common systemic manifestation of Sjögren's syndrome is recurrent joint pain, or arthralgia. Arthritis of the interphalangeal joints of the hands may also occur: they are swollen, painful, and the range of motion in them is sharply reduced. Knee and elbow joints may be affected. As a rule, arthritis is not severe, its symptoms regress after a while, and erosions are not typical.
The skin of people suffering from Sjögren's syndrome is dry and flaky, and these changes are especially pronounced in the area of the upper and lower extremities. When the sweat glands are damaged, sweating decreases.
50% of patients have complaints characteristic of Raynaud's syndrome: high sensitivity to cold, chilliness, numbness of the distal extremities - hands, etc.
In approximately 15% of patients, so-called hyperglobulinemic purpura is diagnosed - pinpoint hemorrhages are visualized on the skin of the lower third of the legs, sometimes in the thighs and/or abdomen; increased skin pigmentation in these areas may be detected.
Very often Sjögren's syndrome occurs with. As a rule, the cervical, submandibular and supraclavicular lymph nodes are involved in the process, and the degree of their enlargement varies from slight to pronounced: in the latter case, the lymph node has the appearance of pseudolymphoma. Malignant ones are also common in this pathology: the likelihood of their development in a person suffering from dry syndrome is 44 times higher than in a healthy person.
If dry syndrome also affects internal organs, this is clinically manifested by symptoms of impaired function: dry, interstitial, and tubular acidosis develop. When the nasal mucosa is damaged, it becomes dry, hard crusts form, and often occur. Olfactory and/or taste sensations deteriorate. When dry crusts close the opening of the nasopharynx, serous otitis media develops, which is manifested by pain in the ear, decreased hearing and moaning of the lesion.
Separately, it is worth mentioning the complaints of patients from the genital organs, the glands of which are often involved in the process of Sjögren's syndrome. Patients are concerned about dryness in the vaginal area, burning sensation in it, and pain during sexual intercourse. Histologically, atrophic changes in the vaginal mucosa, as well as nonspecific vaginitis, are determined. Often these changes are incorrectly regarded as age-related changes in a woman’s body during menopause, and often, on the contrary, are a consequence of it, and dry syndrome has nothing to do with it.
When vessels located in the central nervous system are damaged, neurological symptoms occur: meningoencephalitis, hemiparesis, nystagmus, symptoms of damage to cranial, and more often trigeminal, nerves.
Often, against the background of the pathology we describe, patients develop increased individual sensitivity to certain drugs, in particular to non-steroidal anti-inflammatory drugs, some antibiotics (penicillin), so-called basic therapy drugs, and cytostatics.
Diagnosis of Sjögren's syndrome
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Based on the patient’s complaints, the history of his illness and life, taking into account the results of an objective examination, the doctor will suspect that the patient has Sjögren’s syndrome and will prescribe a number of additional tests to clarify the diagnosis:
- biochemical blood test (hyperglobulinemia);
- immunological blood test (rheumatoid factor classes IgM, IgG, IgA, antibodies to components of cell nuclei, anti-Ro antibodies, anti-La antibodies, antibodies to native DNA, organ-specific and tissue-specific antibodies, immune complexes);
- scintigraphy of the salivary glands;
- sialography of the parotid glands;
- salivary gland biopsy.
Treatment of Sjögren's syndrome
The directions of therapy for dry syndrome are to directly alleviate its symptoms while simultaneously treating the underlying autoimmune disease.
Medicines from the following groups can be used:
- (prednisolone): the regimen and dose of the drug depend on the severity of the disease and the presence and severity of systemic manifestations.
- Cytostatics (chlorbutin, cyclophosphamide, azathioprine): usually used in combination with glucocorticoids.
- Aminoquinoline drugs: Delagil, Plaquenil.
- Proteolytic enzyme inhibitors: Contrical, Trasylol.
- Direct anticoagulants: Heparin.
- Angioprotectors: Solcoseryl, Xanthinol nicotinate, Parmidine, Vazaprostan, used if available
Most often, this syndrome accompanies a number of autoimmune pathologies of connective tissue - dermatomyositis, scleroderma, and in such cases is called secondary Sjögren's syndrome. If the pathology develops independently, then the name sounds like primary Sjogren's syndrome, or Sjogren's disease.
What it is?
Sjögren's syndrome is an autoimmune systemic lesion of connective tissue, manifested by the involvement of the exocrine glands, mainly salivary and lacrimal, in the pathological process, and a chronic progressive course.
Pathomorphology
The main morphological feature is the infiltration of the exocrine glands with lymph and plasma cells. First of all, the lacrimal and salivary glands are affected, a little less often – the glands of the bronchi, digestive tract and vagina.
- Both large and small glands are affected. At first, at an early stage of the disease, only small ducts are involved in the process; as it progresses, its infiltrate spreads further to the gland tissue itself, as a result of which the glandular tissue atrophies and is replaced by connective tissue. In some cases, the infiltrates described above occur not only in the exocrine (external secretion) glands, but also in other organs and systems of the body, in particular in the muscles, lungs and kidneys. This ultimately leads to dysfunction of the affected organ.
- In 30-40% of patients, in the material taken by biopsy of the salivary glands, metaplasia (modification) of the cells lining the ducts is determined: myoepithelial islands appear.
The lobules of the affected glands are destroyed in some patients, while in others the lobular structure is preserved. The glands are either enlarged in size or within normal limits.
Interestingly, even in the absence of clear clinical symptoms of Sjögren's syndrome, a patient with any connective tissue disease will most likely show histological signs of inflammation of the salivary glands.
Reasons for development
The causes of Sjögren's syndrome have not yet been fully established. Among the most likely is the theory of a pathological reaction of the body's immune system. This reaction develops in response to damage to the cells of the external glands by a retrovirus, in particular the Epstein-Barr virus, herpes virus VI, cytomegalovirus, and human immunodeficiency virus. Despite the significant similarity of immunological disorders with changes in the body affected by the virus, direct evidence of the role of the virus as the cause of the development of pathology has not been obtained.
The viruses themselves and the epithelial cells of the glands changed by their influence are perceived by the immune system as antigens (foreign agents). The immune system produces antibodies against such cells and gradually causes the destruction of gland tissue. The disease often occurs as a hereditary or familial disorder, particularly among twins, suggesting a genetic predisposition.
Thus, it is assumed that in the mechanism of development and occurrence of pathology, a combination of many factors is important:
- stress reaction of the body, which occurs as a result of the immune response;
- immune regulation with the participation of sex hormones, as evidenced by the rare incidence among people under 20 years of age, while girls are most often affected among children;
- immune control using T lymphocytes;
- viral;
- genetic.
There are two types of Sjögren's syndrome: primary, where symptoms of the disease are the first manifestations of it, and secondary, when symptoms appear in patients suffering from other rheumatic diseases, such as scleroderma, rheumatoid arthritis or systemic lupus erythematosus. Primary and secondary variants of the syndrome occur with approximately the same frequency. This sometimes makes it difficult to make an accurate diagnosis. Sjögren's syndrome is quite common: in the UK, for example, there are about half a million sufferers. Women aged 40 to 60 years are most often affected, while only one patient in 13 is male.
Symptoms of Sjögren's syndrome
Symptoms of the disease are divided into two groups:
- Glandular manifestations in which epithelial glands are affected and their functions are disrupted.
- Extraglandular manifestations. The symptoms of this group vary greatly, due to damage to various human organs.
Glandular symptoms include the following:
- Pathology of the lacrimal glands. In this case, unpleasant and painful sensations occur in the eyes (burning, as if the eyes are starting to sting, a sensation of sand in them), and characteristic itching and redness appear near the eyes. As a result, vision decreases, pinpoint hemorrhages, swelling, sensitivity to light, and eye pain occur.
- Pathology of the salivary glands. The manifestation of inflammation is mainly of the parotid glands, which are increased in size, sometimes with painful sensations. Dryness of the oral mucosa also occurs, swallowing food is difficult, often patients have to wash down food with water due to the problem of swallowing. In this case, the mucous membrane of the mouth takes on a bright pinkish tint.
- Dryness of the respiratory tract, which leads to inflammation of the bronchi, trachea, and lungs.
- Skin disorders resulting in dry skin.
- Damage to the nasal mucosa. The occurrence of dryness in the nose, covering with an intranasal crust, as a result of which inflammation develops.
Extraglandular manifestations are characterized by the following signs:
- The occurrence of tracheobronchitis, accompanied by cough and shortness of breath. It is not uncommon for a patient to be examined for pneumonia or pulmonary fibrosis.
- Enlarged lymph nodes.
- Damage to the peripheral nervous system. At the same time, painful sensations (tingling, burning) begin to appear.
- Fever;
- Pain in joints and muscles.
- Damage to the thyroid gland. It is a rare disorder in which allergic reactions to various foods, medications and other drugs begin to appear.
- Enlarged lymph nodes (submandibular, cervical, occipital). Enlargement of the liver and spleen is often observed.
- Inflammation of blood vessels, which occurs as a result of atherosclerosis of the lower extremities, due to circulatory disorders. When the functioning of blood vessels is disrupted, skin diseases (rashes) appear, which are accompanied by itching, burning and fever.
Often, against the background of the pathology we describe, patients develop increased individual sensitivity to certain drugs, in particular to non-steroidal anti-inflammatory drugs, some antibiotics (penicillin), so-called basic therapy drugs, and cytostatics.
Diagnostics
Burning eyes and dry mouth may not always mean that a person has this syndrome. Sjögren's syndrome can be diagnosed only in the presence of inflammatory lesions of the glands. However, there are cases when various metabolic diseases can lead to a similar result, in which the secretion of saliva is significantly reduced (most often it is diabetes mellitus).
As a result, the functions of the salivary and lacrimal glands prematurely decline in older people. These forms of dry eyes and dry mouth have nothing to do with Sjögren's syndrome. Tissue examination is also performed to diagnose this syndrome. For this type of study, small fragments of the oral mucosa are taken, which are examined using a microscope. In this way, damage to the mucous glands is established.
Complications
Common consequences of Sjögren's syndrome:
- lymphomas (neoplasms affecting the blood and lymph nodes);
- vasculitis (an inflammatory process in blood vessels that can occur everywhere);
- addition of a secondary infection;
- development of cancer;
- inhibition of blood formation, reduction in the blood of leukocytes, erythrocytes and/or platelets.
If the patient does not take any measures to treat Sjögren’s disease for a long time or he was prescribed the wrong therapy, the pathology progresses, leading to serious disruptions in the functioning of organs and systems.
Treatment of Sjögren's syndrome
In the presence of Sjögren's syndrome, treatment is carried out depending on the stage of the disease and the presence of systemic manifestations.
In order to stimulate the function of the glands, the following is carried out:
- drip administration of contrical.
- subcutaneous administration of galantamine.
- Vitamin therapy courses are conducted for general strengthening purposes.
- As a symptomatic treatment, “artificial tears” (drops in the eyes) are prescribed - with low viscosity - Lakrisifi (200-250 rubles), Natural Tear (250 rubles), medium viscosity Lakrisin, high viscosity Oftagel 180 rubles, Vidisik 200 rubles, Lacropos 150 rubles .
At the initial stages, in the absence of damage to other systems and unexpressed laboratory changes, long courses of glucocorticosteroids (prednisolone, dexamethasone) in small doses are prescribed.
If symptoms and laboratory parameters are significantly pronounced, but there are no systemic manifestations, cytostatic immunosuppressive drugs - cyclophosphamide, chlorobutin, azathioprine - are added to corticosteroids. Maintenance therapy is carried out with the same drugs for several years.
- If there are symptoms of systemic damage, regardless of the stage of the disease, high doses of corticosteroids and immunosuppressants are immediately prescribed for several days with a gradual transfer to maintenance doses.
- For generalized polyneuritis, vasculitis, kidney damage and other severe manifestations of the disease, the following methods are added to the above treatment, extracorporeal treatment - plasmapheresis, hemosorption, plasma ultrafiltration.
- The remaining drugs are prescribed depending on complications and concomitant diseases - cholecystitis, gastritis, pneumonia, endocervicitis, etc.
In certain cases, it is necessary to follow a dietary diet and limit physical activity.
Prevention
To prevent violations, it is necessary to follow all the instructions of the attending physician to prevent exacerbations. You also need to follow a few simple rules:
- limit the load on the organs of vision;
- if the syndrome occurs as a result of another disease, the cause must be treated.
- avoid stressful situations.
Forecast
Sjögren's syndrome can damage vital organs with a transition to a stable state, gradual progression, or, conversely, long-term remission. This behavior is also typical for other autoimmune diseases.
- Some patients may have mild symptoms of dry eyes and mouth, while others develop serious complications. Some patients are completely helped by symptomatic treatment, others have to constantly struggle with deterioration of vision, constant discomfort in the eyes, often recurrent infections of the oral cavity, swelling of the parotid salivary gland, difficulty chewing and swallowing. Constant loss of strength and joint pain seriously reduce the quality of life. In some patients, the pathological process involves the kidneys - glomerulonephritis, leading to proteinuria, impaired renal concentrating ability and distal renal tubular acidosis.
- People with Sjögren's syndrome have a higher risk of developing non-Hodgkin's lymphoma compared to healthy people and people with other autoimmune diseases. About 5% of patients develop some form of lymphoma.
In addition, it has been found that children of women with Sjogren's syndrome during pregnancy have a higher risk of developing neonatal lupus erythematosus with congenital heart block.