Treatment of Sjögren's syndrome

Causes of Sjögren's syndrome

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

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

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

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

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

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

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

In the early stages of the disease, the salivary, lacrimal and other exocrine glands can increase in size due to the inflammatory process with pronounced infiltration of lymphocytes and plasma cells, concentrated mainly around the ducts. Subsequently, infiltration spreads to the parenchyma of the gland with the development of destructive changes in the acini, their atrophy with replacement by fatty and fibrous tissue, and the gradual formation of secretory insufficiency. In this case, the stroma and partial structure of the glands are slightly damaged.

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

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

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

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

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

Typical complaints are itching, a feeling of dryness, “sand in the eyes,” burning and pain in the eyeballs with strained vision. These unpleasant sensations intensify towards the end of the day due to the evaporation of moisture from the surface of the eyes while they were open. A decrease in lacrimation up to the complete absence of tears is especially noticeable in the case of negative emotions (patients cannot cry) and when exposed to irritating substances.

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

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

The second important, although less specific, symptom is xerostomia. Decreased saliva production is a consequence of chronic parenchymal sialadenitis. Dry mouth may be insignificant for a long time; patients get used to it. Even in the presence of histological signs of sialadenitis, the amount of saliva may remain sufficient for quite a long time so as not to cause obvious discomfort. Sometimes patients notice that after stimulation (chewing lemon, consuming acidic foods), a large amount of thick (stagnant) saliva is released. In typical cases, signs of xerostomia first appear sporadically - during excitement, during a long conversation, and over time they become permanent. The patient is forced to drink liquid while talking, sleeping at night, and eating dry food. Hot and spicy foods cause burning and pain in the oral mucosa. Discomfort and dry mouth are so severe that they make chewing, bolus formation, and swallowing difficult. Taste sensations may change.

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

At a late stage of the disease, saliva is practically not secreted, the patient loses the ability to talk and eat without moisturizing the mouth. Keratosis of the mucous membrane, atrophy of the papillae of the tongue, and teeth fall out occur.

An important sign of chronic sialadenitis is an increase in the parotid salivary glands. It is found in 60% of patients. This increase may be bilateral, unilateral or asymmetrical. In the case of a slow increase, palpation of the glands is painless or slightly painful. In half of patients with mumps, it has a recurrent course; outside of an exacerbation, the enlargement of the glands may be unnoticeable. Recurrences of mumps are accompanied by swelling and tenderness of the glands, difficulty opening the mouth, and trigeminal sensory neuropathy.

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

Characteristic is the development of dry (atrophic) tracheobronchitis, in which thick mucus causes a severe dry cough, sometimes with bronchospasm. Often signs of Sjögren's disease are dry and flaky skin, hyperkeratosis, and decreased sweating. Damage to the apocrine glands of the female external genitalia in 30% of patients is accompanied by dryness and atrophy of the vaginal mucosa. In women during menopause, the phenomena of atrophic colpitis are layered with involutive changes due to estrogen deficiency. It is possible to develop chronic gastritis with secretory insufficiency, up to achylia, and a decrease in the exocrine function of the pancreas. However, in a significant number of patients, hypofunction of these glands is not accompanied by clinical symptoms.

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

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

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

With cryoglobulinemia, hepatitis naturally develops. A slight enlargement of the liver in combination with moderate laboratory signs of impaired liver function is often due to the hepatotoxic effect of drugs used in the treatment of Sjögren's disease. In 20% of patients, lymphadenopathy is detected - regional (enlarged submandibular, cervical, supraclavicular nodes) or generalized. With a targeted X-ray and functional examination, symptoms of interstitial pneumonia can be found in almost half of the patients, but clinically this pathology is quite mild, cough and shortness of breath usually occur sporadically. Recurrences of dry or effusion pleurisy with a small amount of exudate, as a rule, pass quickly and do not require treatment. Interstitial pulmonary fibrosis (fibrosive alveolitis) develops very rarely.

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

There is no cardiac pathology specific to Sjögren's disease. Asymptomatic pericarditis and left ventricular diastolic dysfunction are very rarely diagnosed.

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

How to treat Sjögren's syndrome?

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

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

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

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

Indications for the use of efferent therapy include ulcerative-necrotizing vasculitis, polyneuritis and damage to the central nervous system, cryoglobulinemic nephritis, cryoglobulinemia with monoclonal hyper-β-globulinemia. There is no evidence yet of the effectiveness of anticytokine drugs in Sjögren's disease.

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

If tear secretion is at least partially preserved, minor surgical interventions are performed aimed at blocking tear secretion through the nasolacrimal ducts. In case of secondary infection, antimicrobial and antifungal drugs are prescribed.

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

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

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

What diseases can it be associated with?

Organ damage due to Sjögren's disease affects many organs and systems and is manifested by the following diseases:

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

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

Treatment of Sjögren's syndrome at home

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

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

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

What medications are used to treat Sjögren's syndrome?

In the initial stage, aminoquinoline drugs are prescribed:

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

For systemic lesions:

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

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

• - 1000 mg per 1 m2 of body surface intravenously in 200 ml of isotonic sodium chloride solution or 5% glucose solution over 40-60 minutes for 3 consecutive days;
• - 1000 mg per 1 m2 of body surface is combined with methylprednisolone on the 2nd day.

Treatment of Sjögren's syndrome with traditional methods

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

Treatment of Sjögren's syndrome during pregnancy

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

A woman with Sjögren's syndrome is advised to undergo hospitalization in the early stages to clarify the diagnosis, identify the characteristics of the course of the disease and decide on the possibility of continuing pregnancy and developing treatment tactics.

Which doctors should you contact if you have Sjögren's syndrome?

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

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

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

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

Damage to the minor salivary glands can be proven by examining a biopsy of the mucous membrane of the lower lip. There is no need for a biopsy of large glands, since the processes of infiltration in the small and large salivary glands occur in parallel. Histological signs of damage to the salivary glands in Sjögren's disease and Sjögren's syndrome are the same.

To diagnose parenchymal parotitis, sialography (x-ray examination of the parotid glands after the introduction of a radiopaque substance into the parotid duct), sialometry (assessment of unstimulated and stimulated salivary flow), and scintigraphy (assessment of the rate of salivary secretion) are also used.

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

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

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

Sjögren's syndrome is an autoimmune disease that causes systemic damage to connective tissues. The exocrine glands – 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 salivary and lacrimal.

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 sicca 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 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;
• 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:

1. A general blood test reveals an accelerated ESR, anemia and a low level of leukocytes.
2. OAM is characterized by the presence of protein.
3. Protein is also elevated in the biochemical blood test. In addition, the study reveals the maximum titers of rheumatoid factor.
4. A special blood test for the presence of antibodies to thyroglobulin in 35% shows an increase in their concentration.
5. 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 (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. The drug 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.

The course of the disease can be chronic and subacute; there are initial, severe and late stages of the disease.

Pathological processes occur at these stages with varying degrees of immunological and inflammatory activity.

The disease is widespread among patients of different ages; in 90% of cases these are elderly and middle-aged women.

Causes

The causes and mechanisms of the disease have not been established to date; most often the disease develops due to autoimmune factors. Hereditary predisposition also plays a role; the disease often occurs among close relatives (most often in the female line).

Under the influence of unknown factors, a malfunction occurs in the body, as a result of which the salivary and lacrimal glands are infiltrated by T- and B-lymphocytes.

At the same time, antibodies to the epithelial cells of the affected glands are detected in the blood, rheumatoid factor and other immunological abnormalities are diagnosed.

Symptoms

Symptoms of Sjogren's disease are divided into glandular and non-glandular.

Ferrous. The main symptom of the disease is a decrease in the synthesis of tear fluid; the phenomenon is accompanied by a burning sensation in the eyes, like sand or from small scratches.

At the same time, symptoms such as redness of the eyes, itching of the eyelids, the formation of a white substance and its accumulation in the corners of the eyes appear. After a certain time, photophobia manifests itself, leading to narrowing of the eyes and decreased vision.

The second constant symptom of the pathology is disruption of the functioning of the lacrimal glands, accompanied by dry lips, the appearance of jamming, enlargement of the lymph nodes and salivary glands, stomatitis, and multiple caries.

In the initial stages, dry mouth appears only with increased physical activity and strong emotional arousal.

Gradually, the symptom becomes permanent; to moisturize the oral cavity, there is a need to drink water while eating or when talking. The amount of saliva decreases noticeably, the lips become crusty, and the tongue becomes dry.

Dryness in the nasopharynx provokes the formation of crusts in the ear canal and nose, which without proper treatment leads to deafness or otitis media.

The disease affects the functioning of the gastrointestinal tract, many patients complain of pain in the epigastric region, and a severe form of gastritis is often diagnosed, resulting from a lack of functioning of the endocrine glands. The disease is accompanied by heaviness in the abdomen, nausea, lack of appetite, and belching.

Pathology can provoke hepatitis, chronic cholecystitis, since the bile ducts are affected during the disease. As a result of impaired functioning of the pancreas, life-threatening pancreatitis develops.

Non-ferrous . Symptoms of Sjogren's disease can be very different, most often patients experience general malaise, shortness of breath, weakness, and joint pain. After waking up, due to muscle stiffness, there may be an inability to move quickly; in 50% of cases, diaphragm spasm, cough, and breathing problems are diagnosed.

A rash may appear on the abdomen and lower extremities, accompanied by itching and redness.

Other signs include allergies to animal hair, pollen, chemicals, and another symptom is intolerance to drugs (sulfonamides, painkillers, antibiotics).

Diagnostic methods

Sjögren's disease is an autoimmune disease, the causes of which are not fully understood.

Pathology requires careful diagnosis, the purpose of which is to establish symptoms and select the most appropriate treatment option; a favorable prognosis is guaranteed only if timely, systematic and qualified treatment is carried out.

Diagnosis is made using the following methods:

• Instrumental examination;
• Laboratory data;
• Clinical symptoms.

During the examination, the specialist primarily pays attention to the symptoms and clinical picture.

Instrumental examination methods:

The diagnosis is confirmed by the following laboratory data:

• The presence of inflammatory signs in various blood tests, characterized by accelerated ESR, a decrease in the number of platelets, hemoglobin, erythrocytes, leukocytes, and an increase in the level of gamma globulin;
• Detection of immunological disorders (presence of autoantibodies in the blood).

Treatment is prescribed only after a thorough study of symptoms, laboratory data and instrumental examination.

Treatment

Sjögren's disease is a fairly dangerous disease that can lead to disability and even death. A comprehensive diagnosis of all manifestations will neutralize or reduce the causes of poor health.

The nature of the treatment regimen will depend on the prevalence of certain symptoms. In case of Sjögren's disease, it is recommended to avoid self-medication, which can only complicate the overall picture. At the initial stage, hormonal therapy and low doses of antidepressants are prescribed.

Prednisolone and Chlorbutin are used as maintenance therapy; the drugs can alleviate the patient’s condition for several years without causing addiction.

Such treatment is indicated in the initial stages of the disease with pronounced disturbances in laboratory data on the activity of the process.

Patients with severe manifestations of the disease are prescribed large doses of prednisolone (for 3 days) and cyclophosphamide (once), followed by a transfer to moderate doses of cytostatics (cyclophosphamide) and prednisolone.

In order to preserve vision and combat dry eyes that accompany Sjögren's syndrome, drops are used whose function is to replace natural tears. These can be Oftagel, Vidisik, Artelak, Systane, Artificial tears.

To prevent infections, the eyes must be washed with solutions of chloramphenicol and furatsilin. To improve the condition of the oral mucosa, novocaine injections and drugs that stimulate salivation are prescribed. Therapy also includes the use of the drug Pilocarpine.

Treatment with folk remedies

In the treatment of Sjögren's disease, folk recipes are also used; most often, herbal decoctions (plantain, chamomile, oak bark, sage) are used to rinse the mouth. Essential oils are also used as skin care products, including oil of orange, lavender, rose, flax , argan, coconut.

To eliminate the effect of red eyes, it is recommended to use potato and/or dill juice; cotton swabs should be soaked in freshly prepared juice and applied to the eyes. After the procedure, the eyes need rest; it is not recommended to strain them for two to three hours.

Sjogren's disease in almost all cases is accompanied by disturbances in the functioning of the gastrointestinal tract, so it is necessary to eat properly. Products must undergo careful processing; vitamins, carbohydrates, fats and proteins must be present in the diet in the required amount. It is recommended to eat small portions 5-6 times a day.

– 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 Sjogren'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.

What is it: Sjögren's disease and syndrome? This is a disease accompanied by systemic damage to the connective tissues of the body and the external secretion glands (lacrimal, salivary).

How does a disease differ from a syndrome? According to most authors, the final symptom complex, i.e. The clinical picture of these diseases is identical. The difference lies in the order of occurrence of individual symptoms:

• If the typical manifestations of the disease itself (dry eyes and mouth) develop in the absence of any immune damage, then this is the primary syndrome, or Sjögren's disease. Subsequently, it is often accompanied by secondary autoimmune diseases in the form of systemic lupus erythematosus, rheumatoid arthritis, etc.
• The appearance of a clinical picture against the background of an existing autoimmune disease is called “secondary Sjögren’s syndrome.”

Important! Despite the fact that both the disease and Sjogren's syndrome occur in different age categories, there is a certain risk group. This includes women aged 20 to 60 years, because more than 80% of cases of the disease were registered in them.

Despite the fact that Sjogren's disease (syndrome) has recently been studied very actively due to its wide distribution (in the USA alone this figure is more than 4 million people), the causes and pathogenesis of the disease have not yet been clarified.

Probably the triggering factor is an innate genetic predisposition, because It has been noted that the incidence of Sjogren's disease (syndrome) among girls whose mothers have this pathology is tens of times higher than in the general population.

Pathogenesis (development mechanism) is represented by an autoimmune reaction of the body. This means that the body’s immune cells (defenders) - lymphocytes - undergo a mutation during their development, which leads to the appearance of receptors against their own tissues. Once in the bloodstream, they begin to perceive their cells as foreign and attack them, destroying them.

Classification

There is a division of Sjogren's disease (syndrome) according to the degree of activity of the development of the process:

• Highly active: accompanied by the development of a violent inflammatory reaction in target organs (mumps, conjunctivitis, arthritis, etc.). A typical pain syndrome, external signs of inflammation (redness, swelling, local or general rise in temperature), and changes in the blood appear.
• Moderate activity: accompanied by a picture of an inflammatory reaction, but not as pronounced as in the first case. Signs of destruction (destruction) of organs and tissues also appear.
• Low-active: characterized by the slow development of a process that is destructive in nature. Symptoms of dry eyes, xerostomia, and frequent recurrent diseases of the upper respiratory tract gradually appear. This occurs due to the destruction of the exocrine glands. The inflammatory reaction is minimal.

In addition, it is possible to divide Sjögren's disease (syndrome) according to the type of course:

• subacute;
• chronic.

They also highlight the stages of the process:

• initial period;
• period of pronounced changes;
• late period.

Important! Knowledge of these classifications allows you to determine the type of Sjogren's disease (syndrome) and select the most optimal therapy.

Clinical picture

The symptoms of Sjogren's disease (syndrome) are very diverse, which determines subsequent treatment. However, there are three main signs of this pathology:

• xerostomia;
• dry keratoconjunctivitis;
• the presence of any other autoimmune disease.

The combination of two or more of these signs makes the diagnosis valid.

Xerostomia is a dysfunction of the salivary glands. As a result of autoimmune damage in Sjögren's disease, their size increases.

The glands have a dense, smooth consistency. Slightly painful on palpation.

Important! A stony, nodular surface is not a sign of xerostomia! These changes are characteristic of oncological pathology.

As a result of the development of an autoimmune inflammatory process with subsequent destruction of the glands, effective salivation is disrupted. The main complaint is dry mouth. At first, this symptom appears only in moments of excitement, but as the disease progresses it becomes permanent. There are difficulties with eating and swallowing.

Due to impaired salivation, atrophic processes develop in the oral cavity.

The mucous membrane is dry, the tongue is abundantly folded, the papillae are reduced in size or absent altogether. The lips are covered with crusts and cracks, there are jams. Teeth crumble, multiple caries quickly develops.

As a result of a decrease in local immunity, a secondary infection occurs (candidiasis, bacterial and viral stomatitis, cheilitis).

The second typical disease (syndrome) of Sjögren is keratoconjunctivitis (“dry eye”). Redness of the cornea and eyelids appears, as well as copious thick mucous discharge that accumulates in the corners of the eyes.

Subjectively, the patient complains of itching, burning, and a feeling of “sand” in the eyes. There is a decrease in vision. An increase in the size of the lacrimal glands themselves occurs in less than 5% of patients.

Important! When one of these symptoms appears, it is necessary to take into account the individual characteristics of the patient. For example, a feeling of dry mouth and difficulty swallowing are common in older people. This change is physiological. The symptom of “dry eye” can occur due to improper selection of contact lenses or working at the computer for a long time.

Of the systemic manifestations, the most common is joint damage. Moreover, this can be either the development of rheumatoid arthritis (a concomitant autoimmune disease) or an extraglandular manifestation of Sjögren’s syndrome (disease). Externally, the joints are swollen, enlarged, and the range of motion is sharply reduced.

The joints of the hands are most often affected, less often the knees and elbows. These changes may be periodic.

Some patients (about 15%) have autoimmune vasculitis, which results in pinpoint hemorrhages on the skin.

It is possible that Sjögren's disease (syndrome) may also cause damage to internal organs. May be observed:

• atrophic gastritis;
• myositis;
• polyneuropathy;
• glomerulonephritis;
• bronchitis;
• otitis media, etc.

Also, with this pathology, increased allergic sensitivity to various medications (antibiotics, NSAIDs) develops.

Diagnostics

Often, making a diagnosis is difficult, because... Not many doctors know well what Sjogren's syndrome is, and the abundance of clinical manifestations delays the examination for several months.

In addition to blood tests, instrumental studies are carried out auxiliary:

• sialography and biopsy of the salivary glands;
• histological examination of the mucous membrane, skin, muscles;

Important! When the first symptoms of Sjogren's disease occur, the question arises: which doctor should I contact? The main specialist dealing with this pathology is a rheumatologist.

Treatment

Today, Sjögren's syndrome is treated relatively successfully. There is pathogenetic and symptomatic therapy that facilitates the patient’s general well-being.

Sjögren's syndrome (disease) is treated for life; etiotropic therapy has not been developed.

Symptomatically applied:

• instillation of an “artificial tear” solution for dry eyes;
• rinsing the mouth and softening the mucous membranes with sea buckthorn and rosehip oils;
• Compresses with Dimexide are applied to the salivary glands.

The prognosis for Sjögren's syndrome is not life-threatening, but the quality of life with this disease is greatly affected. That is why it is so important to follow the entire necessary treatment package, because this can significantly improve your well-being and prevent the development of severe complications.