Sjögren's disease and syndrome: symptoms and treatment. Sjögren's syndrome: symptoms, diagnosis, treatment Gun disease
– an autoimmune systemic lesion of connective tissue, characterized by exocrine glandular and extraglandular manifestations. The most common glandular manifestation of Sjogren's disease is a decrease in the secretion of the lacrimal and salivary glands, accompanied by a burning sensation in the eyes and dry nasopharynx. Extraglandular manifestations include myalgia, muscle weakness, arthralgia, hemorrhages, enlarged lymph nodes, neuritis, etc. The diagnosis of Sjögren's disease is made taking into account a complex of clinical and laboratory signs and functional tests. Treatment is carried out with corticosteroid hormones and cytostatics; The course of the disease is often benign.
General information
Sjogren's disease is the leader among collagenoses in terms of frequency of occurrence and develops much more often in women in the age group from 20 to 60 years; In men and children, the disease is less common. The causes of Sjögren's disease are unknown. The most likely predetermining factors are heredity and an autoimmune response to a viral (presumably rotavirus) infection.
The pathogenetic mechanism of Sjögren's disease is the development of an immunoaggressive reaction with the formation of antibodies to one's own tissues and lymphoplasmic infiltration of the ducts of the exocrine glands - salivary, lacrimal, gastrointestinal tract, etc. With the generalized form of Sjögren's disease, a third of patients develop damage to the muscles (myositis), kidneys (interstitial abacterial nephritis), blood vessels (productive-destructive, productive vasculitis), lungs (interstitial pneumonia), etc. Sjogren's disease often occurs together with rheumatoid arthritis, Hashimoto's thyroiditis, systemic lupus erythematosus.
Classification of Sjögren's disease
The course of Sjögren's disease can be subacute or chronic. Taking into account the clinical manifestations and complications, the initial (early), severe and late stages of the disease are distinguished. Pathological processes in Sjogren's disease can occur with varying degrees of inflammatory and immunological activity. A high degree of activity of Sjogren's disease is characterized by clinically pronounced symptoms of mumps, keratoconjunctivitis, stomatitis, arthritis; generalized lymphadenopathy, hepatosplenomegaly, laboratory signs of active inflammation.
The moderately active course of Sjogren's disease is characterized by a decrease in inflammation and immunological activity with a simultaneous tendency to destructive changes in the secreting epithelial glands. With minimal activity of the processes, there is a predominance of functional, sclerotic, dystrophic changes in the salivary, lacrimal, and gastric glands, which is manifested by severe forms of xerostomia, keratoconjunctivitis, and gastritis. Laboratory tests show mild signs of inflammation.
Symptoms of Sjögren's disease
The appearance of eye symptoms in Sjögren's disease is caused by decreased secretion of tears (tear fluid). In this case, patients feel a burning sensation, “scratching” and “sand” in the eyes. Subjective symptoms are accompanied by itching and redness of the eyelids, accumulation of viscous secretions in the corners of the eyes, narrowing of the palpebral fissures, and decreased visual acuity. Dry keratoconjunctivitis develops - inflammation of the cornea along with the conjunctiva of the eye.
The salivary glands increase in size in Sjögren's disease. In a third of patients, as a result of enlargement of the paired parotid glands, a characteristic change in the oval of the face is noted, which in the literature is called “hamster face”. Typical symptoms of Sjogren's disease also include dry lips and oral mucosa, stomatitis, seizures, multiple dental caries (usually cervical localization). If in the early stage of Sjogren's disease dry mucous membranes are noted only during physical activity and excitement, then in the severe period the feeling of dryness is constantly observed, forcing the patient to frequently moisten the mouth and wash down food.
Upon examination, a bright pink color of the mucous membranes, their slight injury upon contact, dry tongue, and a small amount of free saliva of a foamy or viscous nature are revealed. Against this background, the addition of a secondary (viral, fungal, bacterial) infection leads to the development of stomatitis. The late stage of Sjogren's disease is characterized by severe dryness of the oral cavity, leading to swallowing and speech disorders, cracked lips, keratinization of areas of the oral mucosa, folded tongue, and lack of free saliva in the oral cavity.
There is hypofunction of other exocrine glands with symptoms of dry skin, nasopharynx, vulva and vagina, the development of tracheitis, bronchitis, esophagitis, atrophic gastritis, etc. With Sjögren's disease, articular syndrome such as polyarthralgia or polyarthritis, impaired sensitivity of the feet and hands, and neuropathy may be observed trigeminal nerve and facial nerve, hemorrhagic rashes on the limbs and torso, fever, myositis, hepato- and splenomegaly.
Diagnosis of Sjögren's disease
Laboratory diagnostic methods include a general blood test, showing moderate leukopenia, anemia, and accelerated ESR. In blood biochemistry in Sjogren's disease, an increase in the level of γ-globulins, total protein, fibrin, seromucoid, sialic acids, and the detection of cryoglobulins are determined. Immunological reactions reveal an increase in the level of immunoglobulins IgG and IgM; the presence of antibodies to DNA, LE cells, antibodies to the epithelium of exocrine glands, muscles, collagen, etc.; increase in the number of B-lymphocytes, decrease in T-lymphocytes.
In Sjögren's disease, a specific reaction to the Schirmer test is noted - a decrease in tear production in response to stimulation with ammonia is determined. When marking the cornea and conjunctiva with dyes, erosions and dystrophic foci of the epithelium are revealed. For Sjögren's disease, contrast radiography (sialography), biopsy of the salivary glands, ultrasound of the salivary glands, MRI of the lacrimal/salivary glands are performed. To identify complications from other systems, chest radiography, gastroscopy, and ECHO-CG are performed.
Treatment of Sjögren's disease
The leading role in the treatment of Sjogren's disease is given to hormonal (prednisolone) and cytostatic immunosuppressive drugs (cyclophosphamide, chlorobutine) and their combinations (prednisolone + chlorobutine, prednisolone + cyclophosphamide). A combination of methods, systemic antibiotics and antimycotics.
Affected mucous membranes of the oral cavity in Sjögren's disease need to be softened and stimulated regeneration with the help of applications of sea buckthorn and rosehip oils, treatment with ointments (methyluracil and solcoseryl). For secretory gastric insufficiency, long-term replacement therapy with hydrochloric acid, natural gastric juice, and pepsin is carried out; in case of pancreatic insufficiency, enzyme therapy is prescribed: taking pancreatin, etc.
Prognosis and complications of Sjögren's disease
The course of Sjögren's disease is not life-threatening, but it significantly worsens the quality of life. Timely initiation of therapy slows down the progression of pathological processes and preserves patients’ ability to work. Without treatment, complications leading to disability are common.
Primary lesions in Sjögren's disease are often accompanied by secondary infection with the development of sinusitis, recurrent tracheitis, and bronchopneumonia. With systemic lesions, the development of renal failure, circulatory disorders of the brain and/or spinal cord is possible.
Specific prevention of Sjögren's disease has not been developed.
Most disruptions to the functioning of a living organism are provoked by specific external stimuli, but there is a separate category of diseases when our immunity begins to destroy normal cells on a large scale. This indicates a failure directly in the process of recognizing native and foreign cells. Defense forces work against connective tissue cells, causing malfunction of many organs - this is Sjögren's syndrome.
What is Sjögren's syndrome
Sjögren's disease or syndrome is an autoimmune disease that affects connective tissue in various body systems. The syndrome belongs to the group of collagenoses. These are the same type of functional and morphological changes in tissue, mainly in its collagen fibers. Collagenosis is characterized by constant progression and involvement of organs, structures of the musculoskeletal system, skin and blood vessels. Sjogren's disease is recognized as the most common among all collagenoses.
Most often, Sjogren's disease is detected in women 20-60 years old, although mainly after 40, when hormonal levels change due to menopause. The specific causes of autoimmune failure have not been clarified, but doctors identify hereditary predisposition and an aggressive response to infection as the most likely.
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There is a theory that the destruction of native cells by the immune system is caused by various viruses. They act asymptomatically, integrating their proteins into the cell membranes of healthy cells, causing the defense system to perceive them as foreign and attack. This mechanism is the basis of many diseases, but systemic connective tissue disorders have the most autoimmune factors.
Connective tissue acts as the “foundation” for all organs; many metabolic and immune processes are ensured through it. Therefore, connective tissue diseases are classified as very severe diseases that greatly affect a person’s life. If arthritis is localized in the joints, rheumatism in the heart, and ankylosing spondylitis in the spine, then with Sjogren's syndrome the glands of the lymphatic system and mucous membranes are affected.
The disease begins with an aggressive immune response, when antibodies to normal cells appear and infiltration of the glandular ducts begins. The exocrine glands (salivary, lacrimal, vaginal) are mainly affected. When the disease spreads to the entire body, a third of patients are diagnosed with myositis (inflammatory damage to muscle tissue), nephritis (inflammatory destruction of the kidneys), vasculitis (productive or productive-destructive), and pneumonia. Sjögren's disease may be detected by screening for arthritis, Hashimoto's thyroiditis, and systemic lupus.
Forms and stages of Sjögren's syndrome
There are subacute and chronic forms of the disease. For subacute, extraglandular symptoms are more typical: rheumatological disorders, vasculitis and corresponding skin lesions, malfunctions of the kidneys and PNS. Chronic is characterized by a predominance of glandular abnormalities: improper functioning of the glands, diseases of the ENT organs.
According to the severity of symptoms, early, severe and late stages are distinguished. Negative changes in connective tissue occur with varying intensity. With a high degree of intensity, symptoms of mumps, stomatitis, conjunctivitis and arthritis are noted. In severe cases, generalized lymphadenopathy (enlarged nodes of the lymphatic system) and hepatosplenomegaly (enlarged spleen and liver) are diagnosed. Based on the results of laboratory tests, a violent inflammatory process is revealed.
Moderate intensity is expressed in a decrease in the immune response, there is extensive destruction of the epithelium of the glands. Low intensity causes xerostomia (saliva deficiency), (inflammatory damage to the conjunctiva and cornea) and gastritis (inflammation of the gastric mucosa).
Symptoms of Sjögren's disease
At first, a person feels only dry eyes and occasional blurred vision. Over time, these symptoms are accompanied by dry mouth, and the need to drink food appears. The formation of small ulcers in the oral cavity and multiple caries is possible. Without treatment, the symptoms worsen, affecting the genitals, digestive and respiratory systems. Symptoms of gastritis, colitis, bronchitis, as well as muscle discomfort appear. In the advanced stage, nerve damage and Raynaud's syndrome are possible, when a person suffers from chilliness, a feeling of wet limbs and goosebumps.
Symptoms of Sjögren's disease:
- Ophthalmological symptoms. Insufficiency of the lymphatic system is expressed in a decrease in the production of tear fluid. Drying of the mucous membrane is accompanied by burning and itching. There may be redness of the eyelids, accumulation of discharge, and blurred vision. With a long course of the disease, conjunctivitis or keratoconjunctivitis sicca develops. A complication may be perforation of the cornea in the presence of an ulcer. Gradually, dystrophy of the cornea and conjunctiva begins, filamentous keratitis or xerosis (drying of the eye mucosa) develops.
- Symptoms of ENT organs. Characteristic signs of Sjögren's syndrome: pronounced coloring of the oral mucosa, dry tongue, seizures, insufficiency of free saliva, stomatitis, extensive dental damage. At first, discomfort is felt only after physical exertion, but later it persists constantly. Swallowing disorders occur, the tongue becomes covered with folds, and foci of keratinization are visible on the mucous membrane. Secondary infection leads to the development of severe viral, fungal or bacterial stomatitis. Cheilitis and dry nasopharyngolaryngitis are also possible. Involvement of the respiratory system is accompanied by a change in voice and the appearance of hoarseness.
- Lymphatic symptoms. Insufficient functionality of the endocrine glands causes dryness of the skin, nasal passages, pharynx, vulva and vagina. Tracheitis, bronchitis, esophagitis and gastritis may develop.
- Neurological symptoms. If connective tissue is damaged, neuropathy of the trigeminal and facial nerves is possible. The development of generalized vasculitis is reflected in the functioning of the peripheral nervous system, which is expressed in polyneuropathy, mononeuropathy, mononeuritis, radiculoneuropathy.
- Rheumatological symptoms. Articular syndrome occurs as polyarthralgia or polyarthritis. There are regular recurrences of swelling and pain in the joints. 70% of patients with Sjogren's syndrome have arthralgia, and 30% have non-erosive arthritis of the small joints of the hands of a recurrent nature. Possible muscle damage (myositis) and impaired sensitivity of the limbs.
- Skin symptoms. Hemorrhagic rash, increased dryness of the skin, cracks (with a decrease in air temperature). Purpura is observed in 30% of patients (hypergammaglobulinemic in lymphocytic vasculitis and cryoglobulinemic in destructive). Against the background of destructive vasculitis, ulcerations on the legs also occur.
- Other symptoms. Manifestations of the syndrome include hepatomegaly (enlargement and hardening of the liver) and splenomegaly (enlargement of the spleen). Many are diagnosed with interstitial nephritis or glomerulonephritis with nephrotic syndrome.
Damage to the salivary glands in this connective tissue disease occurs as sialadenitis or mumps, less often the symptoms resemble submaxillitis or sialodochitis. Sometimes there is an increase in small glands, much more often the parotid or submandibular glands are affected, which noticeably changes the oval of the face.
Diagnosis of Sjögren's syndrome
The examination begins with examining the patient. The specialist should identify dry skin and tongue, the presence of ulcers on the oral mucosa and multiple caries, inflammation of the eyes and decreased tear production. Systemic damage to the connective tissue causes swelling of the parotid glands. Periodic swelling of the joints is possible, and upon palpation - pain. It is recommended to additionally listen to the lungs for wheezing.
When diagnosing Sjogren's syndrome, you need to consult a therapist, rheumatologist, ophthalmologist, dentist and other specialists. The dentist can clarify the nature of the damage to the salivary glands, excluding diseases with similar symptoms. An ophthalmologist conducts a test to assess the functionality of the lacrimal glands: exposure to ammonia reduces the production of tear fluid. Marking of ocular structures makes it possible to identify erosion and dystrophy.
Laboratory tests to detect Sjögren's syndrome:
- General blood analysis. This syndrome is characterized by severe leukopenia, that is, a reduction in the number of leukocytes. Leukopenia indicates increased immune activity and the presence of anti-leukocyte antibodies. Almost all patients have mild or moderate anemia, and half have an increase in ESR. This is due to dysproteinemic disorders. An increase in CRP is not typical for Sjogren's syndrome (excluding effusion serositis, glomerulonephritis, destructive vasculitis, demyelinating neuropathy, intercurrent infection).
- General urine analysis. When Sjögren's disease is combined with urolithiasis or chronic pyelonephritis, the addition of a secondary infection causes leukocyturia (the presence of leukocytes in the urine). Characteristic signs of kidney damage include the presence of protein, glucose, red blood cells, casts in the urine, as well as a decrease in specific gravity and increased acidity of urine.
- Blood chemistry. There is a high concentration of γ-globulins, an excess of proteins, fibrin, sialic acids and seromucoid. Increased aminotransferase activity and cholestasis at an early stage of disease development may indicate concomitant viral or autoimmune hepatitis, cholangitis, liver or pancreas pathologies. If the total protein concentration increases and an M gradient is detected, additional diagnostics for plasma dyscrasias are necessary.
- Immunological reactions. Rheumatoid factor is detected in all patients, and its high titers indicate the presence of cryoglobulinemic vasculitis and the formation of MALT tissue. Cryoglobulins are detected in 1/3 of patients. Sjögren's syndrome is characterized by the following picture: an excess of IgG/IgA and IgM antibodies, the presence of antibodies to DNA, collagen and epithelium, the presence of lupus cells, an excess of B-lymphocytes and a lack of T-lymphocytes. Almost all have autoantibodies to nuclear antigens (Ro/SS-A and La/SS-B).
Unfavorable signs in Sjögren's syndrome include a decrease in complement C4, which signals the active development of cryoglobulinemic vasculitis. A sharp increase in the concentration of IgM immunoglobulins may indicate the concomitant development of lymphoma. If a patient has autoantibodies to thyroglobulin and thyroid peroxidase, it is necessary to diagnose autoimmune thyroiditis. In case of chronic liver dysfunction, tests reveal antimitochondrial antibodies, as well as antibodies to smooth muscles and microsomes (liver and kidney tissue).
Additional examination methods:
- sialography or contrast radiography;
- biopsy and ultrasound of the salivary glands;
- MRI of glands;
- X-rays of light;
- gastroscopy (checking the functioning of the esophagus and stomach);
- echocardiography (checking the functioning of the heart).
Ultrasound examination of the salivary glands makes it possible to assess their volume and structure, as well as identify inflammation, cysts and stones in the ducts. Magnetic resonance imaging provides the greatest amount of information about the functioning of the salivary glands. Chest X-rays are often uninformative. Computed tomography of the chest is effective only at an early stage of the disease, when the respiratory system is involved in the inflammatory process. Ultrasound examination of the abdominal organs can identify the causes of dry mouth and throat.
Patients with Sjögren's syndrome should visit a rheumatologist, ophthalmologist, and dentist regularly. It is advisable to see a gastroenterologist, since 80% of people with this disease also suffer from gastrointestinal disorders. At risk are people with a long history of Sjogren's disease and concomitant diffuse atrophic gastritis.
Basic and symptomatic treatment of Sjögren's syndrome
A rheumatologist examines patients and prescribes therapy for Sjogren's syndrome. The process may require consultation with a nephrologist, ophthalmologist, gynecologist, gastroenterologist, pulmonologist and other specialists. Therapy is based on hormones and cytostatic agents with immunosuppressive effects. A combination of Prednisolone with Chlorobutin or Cyclophosphamide is effective.
Symptomatic treatment for Sjogren's disease consists of preventing secondary infection and eliminating excessive dryness. Most people with this disease manage to live normally, only occasionally it manifests itself more severely and can cause death. When the systemic manifestation of the syndrome is pronounced, glucocorticoids, immunosuppressants and other drugs are added to the prescription.
Symptomatic treatment for Sjogren's disease:
- Extracorporeal hemocorrection for the treatment of concomitant vasculitis, cerebrovasculitis, polyneuritis or glomerulonephritis. Depending on the circumstances, plasmapheresis, hemosorption or plasma filtration are performed.
- Artificial saliva and oil solutions to moisturize and restore the oral mucosa. Blockades with novocaine, calcium preparations and ointments (Methyluracil, Solcoseryl) are effective.
- Artificial tears and regular eye rinsing with antiseptic agents. It is possible to use soft contact lenses to prevent dystrophy.
- Medicinal applications with antimycotics (antifungal drugs) or antibiotics against inflammation in the parotid glands.
- Enzyme therapy for malfunctioning pancreas. Adequate prevention of diabetes mellitus.
- The use of hydrochloric acid and gastric juice for gastric insufficiency. Pepsin is also used.
- Diet and additional consumption of yoghurts with lactobacilli to prevent stomatitis, vaginitis and similar complications.
- Daily use of fluoride gels to protect dental tissue.
To moisturize the eyes, you can use medications (drops and gels) sold in pharmacies without a prescription. Eye comfort is also ensured by increasing humidity in home and work areas. The patient is not recommended to sit near heaters and air conditioners. On the street you need to wear glasses and protect your eyes from solar radiation in every possible way.
To moisturize the oral cavity, you need to drink a lot of clean water or spray a glycerin solution into your mouth (20 ml of product per 1 liter of water). You can stimulate saliva production with water and lemon juice, lollipops, or regular chewing gum. It is important that these products do not contain sugar or harmful ingredients. Warm saline solutions help to painlessly moisturize and cleanse the nasal passages.
To eliminate vaginal dryness, there are various hypoallergenic suppositories, ointments and gels. It is better to ask your gynecologist for a prescription for the drug during a routine examination, so as not to worsen the situation. If the skin is hypersensitive, it is not recommended to take a hot shower or bath and wipe dry with a rough towel. After water treatments, you can apply a moisturizer or special oil to the skin to retain moisture.
Prognosis and possible complications
Sjögren's syndrome does not pose a serious threat if treated correctly, but its manifestations can complicate a person's life. It is necessary to eliminate symptoms in a timely manner and carry out supportive procedures so that a person does not become disabled due to severe complications. Only through complex treatment can it be possible to slow down the destruction of connective tissue and maintain a person’s ability to work.
Complications and consequences of Sjogren's syndrome:
- secondary infection;
- mumps;
- sinusitis;
- blepharitis;
- bronchitis;
- bronchopneumonia;
- tracheitis;
- diabetes;
- kidney failure;
- thyroid dysfunction;
- circulatory disorders in the brain.
Prevention of Sjögren's disease
There is no specific prevention against connective tissue diseases, since medicine has not identified the exact causes of the development of the disease. Doctors have proposed a list of general measures that will help reduce the risk of developing the disease or detect it at an early stage. The main role in this list is given to the prevention of infection, especially rotavirus infections. Women over 40 and people with a family history of Sjögren's syndrome need to take prevention seriously.
Basic preventive measures:
- regular visits to specialists, contacting doctors for symptoms;
- prevention and treatment of infectious diseases, especially ENT infections;
- treatment and control of all associated disorders;
- taking all medications as prescribed by the doctor;
- increasing stress resistance, avoiding emotional overload;
- limiting stress on the visual system, speech and digestive organs;
- timely treatment to the hospital in case of exacerbation;
- refusal of any type of tanning and radiation therapy;
- physiotherapeutic procedures and vaccinations only after the approval of the attending physicians.
Sjögren's syndrome is common and dangerous. The disease is accompanied by many unpleasant symptoms and requires constant treatment with drugs that suppress the immune system. Only the joint efforts of a rheumatologist, dentist, ophthalmologist and gastroenterologist can maintain a stable condition and performance in Sjögren’s disease.
Sjogren's disease is a systemic autoimmune disease related to connective tissue diseases; characterized by damage to 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.
Sjogren's disease is the most common disease among connective tissue diseases and occurs in women 10–25 times more often than in men, usually between the ages 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 Sjogren's disease can be divided into glandular and extraglandular.
Glandular manifestations are caused by 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 decreased secretion of tear fluid. Patients complain of burning sensations, “scratching” and “sand” in the eyes. Often there is itching of the eyelids, redness, and accumulation of viscous white discharge in the corners. Later, photophobia, narrowing of the palpebral fissures appear, and visual acuity decreases. Enlargement of the lacrimal glands in Sjögren's disease is rare.
The second mandatory and constant sign of Sjogren's disease is damage to 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, and multiple (usually cervical) dental caries are noted. In a third of patients, a gradual enlargement of the parotid glands is observed, leading to a characteristic change in the oval of the face, described in the literature as a “hamster face” or “chipmunk face.”
In the initial stage of the disease, dry mouth appears only during physical activity and anxiety.
In the severe stage, dry mouth becomes constant, accompanied by the need to wash down dry food and the desire to moisten the mouth during a conversation. The oral mucosa becomes bright pink and is easily injured. There is little free saliva, it is foamy or viscous. The tongue is dry. The lips are covered with crusts, inflammation is observed, and a secondary infection, including fungal and viral, may occur. Multiple cervical dental caries is characteristic.
The late stage is manifested by severe dryness of the mouth, the inability to speak, or swallow food without washing it down with liquid. The lips of such patients are dry, cracked, the oral mucosa shows signs of keratinization, the tongue is folded, and free saliva in the oral cavity is not detected.
Dryness of the nasopharynx with the formation of dry crusts in the nose and in the lumen of the auditory tubes can lead to temporary deafness and the development of otitis media. Dryness of the pharynx and vocal cords causes hoarseness.
Frequent complications are secondary infections: sinusitis, recurrent tracheobronchitis and pneumonia. Damage to the glands of the external genitalia is observed in approximately 1/3 of patients with Sjogren's disease. The vaginal mucosa is reddened, dry, and patients are often bothered by burning pain and itching.
A common symptom of Sjögren's disease is dry skin. Sweating may be reduced.
Swallowing problems are caused by 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 of air, nausea, and loss of appetite. Pain in the epigastric region is less common.
There is a direct relationship between the degree of dryness and inhibition of the secretory function of the stomach. Damage to the bile ducts (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, and poor tolerance to fatty foods.
Involvement of the pancreas in the process (pancreatitis) is manifested by pain and digestive disorders.
Extraglandular manifestations of Sjögren's disease are very diverse and systemic in nature. Joint pain, slight stiffness in the morning. Signs of muscle inflammation (muscle pain, moderate muscle weakness, 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 the submandibular, cervical, occipital, supraclavicular lymph nodes, and in 1/3 of patients the enlargement of the lymph nodes is widespread. In the latter case, liver enlargement is often detected.
Various respiratory tract lesions are observed in 50% of patients. Dry throat, soreness and scratching, dry cough and shortness of breath are the most common complaints.
In Sjögren's disease, vascular damage is noted. Pinpoint bloody rashes most 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. The rash is accompanied by itching, painful burning and increased skin temperature in the affected area.
Damage to the nervous system with sensory disturbances of the “socks” and “gloves” type, neuritis of the facial and trigeminal nerves are observed in a third of patients.
A third of patients experience 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%). Cryoglobulins are detected in a third of patients.
In outpatient settings, it is advisable to take into account various combinations of the following signs:
- 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, mainly cervical, dental caries;
- recurrent chronic conjunctivitis;
- persistent increase in ESR (over 30 mm/h);
- hypergammaglobulinemia (over 20%);
- presence of rheumatoid factor in the blood (titer more than 1:80).
Although none of these signs, taken individually, is specific for Sjögren’s disease, the presence of four or more signs allows in 80–70% of cases to suspect and subsequently confirm the diagnosis using special research methods.
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 Sjogren'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 abnormalities in laboratory parameters, long-term treatment with prednisolone in small doses (5–10 mg/day) is advisable.
In 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 chlorobutine (2–4 mg/day), followed by long-term, for several years, maintenance doses of prednisolone (5 mg/day). day) and chlorbutin (6–14 mg/week).
This regimen can be used to treat patients in the initial stages of the disease in the presence of severe disturbances in 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 days in a row and a single intravenous injection of 1000 mg of cyclophosphamide), followed by transfer to moderate doses of prednisolone (30–40 mg/day) and cytostatics (chlorobutin 4- 6 mg/day or cyclophosphamide 200 mg intramuscularly 1–2 times a week) in the absence of effects on the liver is the most effective treatment for patients with severe systemic manifestations of Sjögren's disease, is generally well tolerated by patients and avoids many of the complications associated with long-term use high doses of prednisolone and cytostatics.
Extracorporeal treatment methods (hemosorption, cryoadsorption, plasmapheresis, double plasma filtration) in combination with pulse therapy are most effective in the treatment of patients with Sjogren's disease with ulcerative-necrotizing vasculitis, glomerulonephritis, polyneuritis, myelopolyradiculoneuritis, cerebrovasculitis, caused by cryoglobulinemia.
Local therapy for eye damage is aimed at eliminating dryness and preventing secondary infection. Dry eyes are an indication for the use of 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.
To protect the cornea, therapeutic soft contact lenses are used. To prevent secondary infection, use solutions of furatsilin (at a dilution of 1:5000), 0.25% solution of chloramphenicol, ciprofloxacin, etc.
Therapy for chronic inflammation of the salivary glands is aimed at overcoming dryness, strengthening the walls of the salivary gland ducts, preventing exacerbations, improving the restoration of the epithelium of the oral mucosa and fighting secondary infections.
In order to normalize nutrition and secretion of the salivary glands, novocaine blockades are used. In cases of exacerbation of chronic parotitis (and to prevent its relapses), applications of a 10–30% dimexide solution are used. In cases of the development of purulent mumps, antibiotics are injected into the ducts of the salivary glands and antifungal drugs (nystatin, levorin, nystatin ointment) are prescribed locally. To reduce the permeability of the ducts, calcium preparations are administered intravenously or intramuscularly.
It is possible to accelerate the healing of the mucous membrane of the oral cavity and the red border of the lips when erosions and cracks occur with the help of rosehip and sea buckthorn oils, solcoseryl and methyluracil ointments, as well as by treating the oral mucosa with ENKAD (active nucleic acid derivatives). Decamine caramel also has antibacterial properties.
For dry nasal mucosa, frequent applications of isotonic sodium chloride solution are used (using turundas).
Vaginal dryness is reduced by using potassium iodide jelly.
Forecast
The prognosis for life is favorable. With timely treatment, it is possible to slow down the progression of the disease and restore the ability of patients to work. When treatment is started late, severe manifestations of the disease usually develop quickly and the patient becomes disabled.
What is Sjögren's syndrome, what are the symptoms and treatment this pathology? Disease expressed in defeat salivary or lacrimal glands , their secretion decreases, and the mucous membrane becomes drier. More often, the disease manifests itself in women during menopause. Why is it dangerous? Sjögren's disease , and how to deal with it?
Etiology
Scientists have not yet been able to obtain accurate information about the reasons for the development of pathology. The most likely provoking factor Sjögren's syndrome , a viral infection occurs.
The following types of pathogens take part in pathological processes:
- cytomegalovirus;
- HTLV 1 (lymphotropic virus);
- EBV (Epstein-Barr virus);
- enteroviruses (Coxsackie viruses);
- immunodeficiency virus.
As a result of a number of studies, it was found that Sjögren's syndrome affects people with a genetic predisposition. Most often, women over 40 years of age suffer from the pathology, but the disease is detected in patients at a younger age.
Sjögren's syndrome is a process that develops with the following disorders in the body:
- ovarian hyperfunction;
- lupus erythematosus (erythematous);
- scleroderma vascular lesions;
- nodular vasculitis (periarteritis);
- Besnier Beck Schaumann disease (sarcoidosis).
Sjögren's syndrome (SS) also develops due to the following provoking factors:
- nervous tension;
- psychological trauma;
- hypothermia;
- severe fatigue.
About 15% of patients suffer from SS due to collagen diseases and rheumatoid arthritis. But in most cases the reason is autoimmune lesions of the body.
Pathogenesis
The disease was named after the Swedish ophthalmologist Henrik Sjögren, who first described it.
There is an excessive concentration of elements in the lesions:
- immunoglobulins;
- T and B lymphocytes;
- cytokines that stimulate class II antigens.
For this reason, there is a decrease in the level of T-suppressor compounds and activation of B cells. Due to the predominance of antigens, an autoimmune reaction is formed in relation to the exocrine glands, which ultimately leads to their atrophy and the process of replacement with connective fatty tissue.
The classification is based on the causes of the pathology, therefore there are two types of disease:
- A syndrome that develops together with or against the background of autoimmune diseases, it is also called Sjögren’s symptom . It is more correct to say “syndrome”, because by this we mean a collection of different symptoms.
- Primary pathology, independent of third-party or accompanying processes, formed individually - Sjögren's disease.
The difference also lies in the fact that in the first case the lesion is accompanied by keratoconjunctivitis of the eyes and dryness of the salivary glands. Secondary Schengen syndrome causes sialadenitis and xerostomia. The second type of pathology is rarely observed in children.
Despite some differences, the pathogenesis and etiology are similar.Sjögren's disease and syndromedevelops for the same reasons and patterns.
Clinical picture
Signs of dry mucous membranes are divided into two groups. When it just startsSjögren's syndrome, symptomspoorly expressed. With further development, manifestations depend on the specifics.
Chronic symptoms
Signs are expressed in a decrease in the secretory functions of the glands.Symptoms of Sjögren's disease the following:
- burning in the eyes;
- redness;
- itching sensation in the eyelids;
- photophobia and loss of quality of vision;
- narrowing of the eyes.
Often patients do not understand the appearance of pinpoint infiltrates and minor hemorrhages in the eyes duringSjögren's syndrome - what is it,a harbinger of severe complications or a signal that the body is coping? Such signs are caused by the addition of a staphylococcal infection, which results in suppuration of the cornea with further perforation. Hemorrhages indicate the progression of the disease, and not a decrease in its manifestations.
Systemic symptoms
- development of an inflammatory process in the mouth;
- education jammed;
- dryness of the membranes and lips;
- enlargement of nearby lymph nodes;
- swelling of the parotid glands;
- foamy and scanty salivation.
If in the initial stage dryness appears at the moment of excitement or physical activity, it intensifies laterSchengen syndrome, symptomsin which they are expressed in a change in the shade of the mucous membrane. It turns bright pink and is easily injured.
The tongue also constantly dries out, becomes covered with cracks, and the lips become crusted. Multiple ulcers form in the mouth, merging into lesions.
If the pathology develops further, complications begin. Sjogren's disease is something that leads to serious consequences if left untreated. During an exacerbation, systemic symptoms intensify, and it becomes difficult for a person to speak and swallow. Partial or complete loss of teeth occurs. Foci of keratinized epithelium appear on the mucosa.
Sjögren's disease leads to the fact that the patient’s face takes on shapes similar to the muzzle of a hamster. During remission, enlarged glands return to their original state, but during relapses they change again. In some patients, this manifestation develops gradually and then persists for a long time.
Complications of Sjögren's syndrome
Sjögren's disease affects other organs:
- Generalized enlargement of lymph nodes leads to vasculitis and atherosclerosis of the lower extremities.
- Digestive system: hepatitis and liver cirrhosis develop. Due to the decrease in acidity, ulcers form on the gastric mucosa. A person suffers from nausea and bitterness in the mouth.
- For Sjögren's pathology The vaginal glands are also affected. There is no hydration, sexual intercourse is difficult.
- Due to insufficient sweating, the skin suffers. The skin becomes dry and peels. Body temperature rises and ulcers form.
- Plasticity in the limbs disappears. The joints lose the ability to rotate, movement causes pain, which persists even at rest.
In places where the glands are damaged, malignant formations form. With reduced immunity, there is a high risk of developing cancer.
Diagnostics
Diagnosis is difficult due to mild symptoms at the initial stage of the disease. Three specialists take part in the examination:
- ophthalmologist;
- dentist;
- rheumatologist.
Diagnostic methods for Sjögren's syndrome were developed at the Institute of Rheumatology of the Russian Academy of Medical Sciences.
The criteria include the following factors:
- confirmation of xerostomia and xerophthalmia;
- decreased tear production using the Schirmer test;
- staining of ocular mucous membranes with fluorescein;
- the presence of parenchymal parotitis;
- focal diffuse infiltration of the salivary glands;
- signs of autoimmune pathology and rheumatoid factor.
Also during diagnosis The following technologies are used:
- sialogram;
- lip tissue biopsy;
- sialometry;
- blood sampling for positive antibodies;
- examination of salivary biopsy.
Laboratory tests are performed to confirm the diagnosis. Early examination helps prevent complications. When differentiating, the development of autoimmune deficiency is excluded.
Therapy for Sjögren's syndrome
For patients suspected of having Sjögren's disease, treatment is developed based on tests and tests. Therapy is complex, depending on the stage of the disease and the presence or absence of complications. At the initial stage, it is customary to treat SS with small doses of corticosteroids, but provided that other systems are not affected:
- Prednisolone;
- Budesonide;
- Kenalog;
- Dexamethasone and others.
With pronounced manifestations successful treatment is carried out with a cytostatic drug with alkylating properties - Chlorambucil. For severe systemic symptoms, pulse therapy is used with parenteral use of a combination of drugs Prednisolone and Cyclophosphamide.
For complications and final stagesSjögren's disease is currently treated successfullyaccording to the following scheme:
- For xerostomia, increased fluid intake is recommended. You need to drink water often. It is necessary to avoid compositions that increase dryness.
- The use of artificial salivary and tear fluid and lubricants: Lakrisin, Visitik and others.
- When a secondary infection occurs, antibacterial therapy is carried out in combination with antifungal agents.
- Vitamin complexes as a general strengthening treatment.
The dentist monitors the condition of the oral cavity, prevents and treats caries, can tell the patient what Sjogren's syndrome is and prescribe general and local medications.
If the pathology has affected other systems, therapy is based on the specifics of the complications. Sometimes dieting and reducing physical activity are recommended.
Other treatment methods
In cases where persistent remission is observed, furtherTreatment with folk remedies for Sjögren's disease:
- To enhance the secretion of saliva, food includes a large amount of stimulating foods: lemon, onion, mustard.
- To avoid stress on the digestive organs during the treatment period, you should avoid fatty, spicy and salty foods. But it is necessary to ensure that important micro- and macroelements are present in the diet. Small and frequent portions contribute to better and safer digestion.
- To relieve eye strain, wash and lotion with dill and potato juice.
- The mouth should be rinsed with herbal decoctions based on chamomile flowers, sage, and oak bark.
Symptoms of Sjogren's disease with proper treatmentcan be stopped in less than a month. The pathology is not particularly dangerous, but it reduces the quality of life and leads to disability. As an autoimmune disease, it moves into stable states, characterized by both long-term progression, with damage to vital organs, and long-term remission.