Sjogren syndrome is a pathology of an autoimmune nature characterized by symptoms of increased dryness of the eye and oral mucous membranes.
This is one of the most frequent diseases of the connective tissue, which occurs mainly in women aged 20 to 60 years. Very rarely, the disease is fixed in young children.
Sjogren's syndrome is incurable, so the patient must provide regular care for problem areas. It is worth noting that the pathology is not accompanied by significant disruptions in the body that can lead to a sharp decline in the quality of life.
Pathology has its own classification by cause. According to this gradation, there are 2 forms of this state:
- Sjogren syndrome, which is manifested in conjunction with other pathologies of autoimmune etiology.
- Sjogren's disease, which occurs as the main disease.
According to the principle of origin and further course, such a process happens:
- Chronic. Differs sluggish progression, due to the effect on the glands. It proceeds without severe symptoms, but is manifested by prolonged malaise of a general nature.
- Subacute. This form of the disease is characterized by a sharp manifestation and damage to various internal organs.
Causes and Risk Factors
The exact reasons for the development of this violation have not yet been studied, so all the allegations regarding this issue are based on the basis of many years of observation by doctors. It is noted that the appearance of Sjogren syndrome is usually associated with a negative effect on the body of numerous factors.
When the immunity is activated, the disease immediately enters the active phase of development. This process is caused by disorders of the regulation of B-lymphocytes in the blood and hypersensitivity reactions. All this may be accompanied by necrosis, degenerative processes or atrophy of the acinar glands, or a decrease in the activity of the lacrimal and salivary glands due to the defeat of the exocrine glands.
As a result, pathological lesion of nerve fibers occurs, provoking the drying of mucous membranes.
With regard to specific causes, Sjogren syndrome can be a consequence of:
- severe overheating or hypothermia;
- long-term use of high doses of drugs;
- psycho-emotional disorders;
- mental overstrain, which is the strongest stress for the body;
- the progression of other diseases of autoimmune etiology;
- hypersensitivity reactions to various substances;
- hereditary factor.
Autoimmune processes develop due to the lack of compensation for the basic needs of the human body. Genetic predisposition to this disease is also explainable, since there is the ability of genes to encode certain reactions to specific influences, stimuli, factors.
Sjogren's syndrome is diagnosed in 5 to 10% of male patients. All other cases occur in women, less often in children. First of all, this is due to the peculiarities of the hormonal background.
Also, this pathology can develop in individuals with:
- viral patolgia of different etiology;
- rotavirus infections;
- herpetic diseases.
But science has not yet been able to prove such an interconnection, therefore these statements are so far perceived only as theories.
Sjogren's disease is considered a severe pathology that requires early detection and adequate treatment. In addition to the glands of internal and external secretion, with this pathology problems can arise with:
- the kidneys;
- the liver;
- thyroid gland;
Symptoms of Sjogren's disease
With a decrease in the secretion of tears, dry mucous membranes of the eyes. The patient complains of discomfort, burning, "sand" in the eyes. Among the subjective symptoms of Sjogren's syndrome, the appearance is noted:
- hyperemia of the eyelids;
- accumulations of viscous fluid in the corners of the eyes;
- narrowing of the palpebral fissures;
- blurred vision.
Against this background, keratoconjunctivitis, a disease accompanied by inflammation of the cornea and conjunctiva, develops.
With Sjogren's disease, an increase in the salivary glands is observed. In one third of patients, due to the increase in the paired parotid glands, the face shape changes noticeably.
In addition, the symptoms of this pathology include the appearance of:
- dryness of the mucous membranes of the mouth and lips;
- extensive caries.
At the initial stage, dryness of the mucous membranes is observed only with strong agitation or intense physical exertion. But then it becomes permanent, so the patient often has to moisten the mouth or drink food.
During a visual examination, the doctor notes the bright pink color of the mucous membranes, dry tongue, scanty viscous and foamy saliva. They are easily damaged even with a minor impact, which becomes a favorable environment for the reproduction of pathogenic microorganisms and the development of stomatitis.
At the transition of Sjogren's disease to a late stage of development, the symptoms are expressed:
- severe dryness of the oral mucosa, which leads to dysfunction of the larynx and problems with speech;
- keratinization of individual sections of the mucous membranes of the oral cavity;
- the sharp dryness of the lips with their cracking;
- wrinkling of the tongue;
- lack of free saliva in the oral cavity.
In parallel with this, the functions of other exocrine glands are suppressed, which manifests itself:
- dry skin and nasopharyngeal mucosa;
- frequent recurrences of tracheitis;
- the development of bronchitis, GERD, atrophic gastritis, etc .;
- dry mucous genital organs in women.
The disease is characterized by the manifestation of:
- articular syndromes by type of polyarthralgia or polyarthritis;
- disorders of the sensitivity of the feet of the lower limbs and hands;
- inflammatory lesions of the facial and trigeminal nerves;
- hemorrhagic rash on the limbs and body;
Diagnosis of Sjogren's syndrome
Diagnosis of this disease is based on:
- medical history and history taking;
- examination of the mucous membranes;
- general clinical and biochemical blood tests;
- sampling salivary gland tissue for biopsy;
- Schirmer test;
- sialography and sialometry;
- examination by an ophthalmologist;
- Ultrasound of the salivary glands;
Features of the manifestation of the disease in various diagnostic activities:
- In the KLA with this disease there are violations in the form of thrombocytopenia, leukopenia, hypercoagulation, anemia, the presence of rheumatoid factor.
- In blood biochemistry, signs of hypergammaglobulinemia, hyperproteinemia, hyperfibrinogenemia are recorded.
- In the immunogram data, antibodies to CIC, cell nuclei are detected, and immunoglobulins G and M are also detected.
- The Schirmer test is based on the use of special paper, which is placed in the lower eyelid for 5 minutes. After that, the length of the wet zone is measured. In Sjogren's syndrome, it does not reach 5 mm.
- Marking of the cornea and conjunctiva. For this purpose, special dyes are used, with the help of which erosive dystrophic foci are detected on the surface of the eye.
- Sialography This diagnosis is based on the introduction of a special substance into the salivary glands. After that, the X-ray machine takes several pictures where the doctor can identify areas of expansion or destruction of the ducts.
- Sialometry is based on the stimulation of salivation with vitamin C. The test helps to identify the amount of fluid released per unit of time.
- Ultrasound and MRI of the salivary glands provide an opportunity to identify hypoechoic areas in the parenchyma of the salivary gland.
With timely detection and treatment of the disease, the prognosis is relatively favorable. In the absence of a timely response to the symptoms, complications develop and a lethal outcome occurs.
How to treat Sjogren's syndrome
Comprehensive treatment of Sjogren syndrome is aimed at:
- achieving sustained remission of the disease;
- improving the quality of life of the patient;
- preventing the development of life-threatening complications.
Non-drug therapy involves:
- avoidance of factors that can cause dryness of the mucous membranes: a long stay near the air conditioner, cigarette smoke, strong dry wind, prolonged work at the computer, etc .;
- restriction of the use of agents provoking local dryness of mucous membranes (tea, coffee, nicotine);
- frequent fluid intake;
- careful monitoring of oral hygiene (use of fluoride toothpaste, regular check-ups at the dentist);
- use of contact lenses selected by a doctor;
- use chewing gum or mints to stimulate salivation.
Treatment for glandular symptoms is:
- The use of moisturizing eye drops. Usually they are used 3 - 4 times a day, but if necessary, the patient can use every hour. With severe dryness, the doctor may prescribe "artificial tears" of increased viscosity, but they can cause blurred vision, so they should be instilled overnight.
- Use antibacterial eye drops.
- Prescribing artificial saliva based on mucin carboxymethylcellulose: Oral balance gel, Biotene rinse, Salivart, Xialine, etc.
- Conducting local and systemic antifungal therapy. It is necessary because Sjogren syndrome is often complicated by a candidal infection.
- Use of cyclosporin A ophthalmic emulsion with dry keratoconjunctivitis.
- Local use of NSAIDs to eliminate ocular discomfort. But such drugs should be used with caution and not for long, as they can cause corneal damage.
- Appointment of GCS in the recurrence of dry keratoconjunctivitis. The course of such treatment should not last more than 14 days.
- Stimulation of the secretion of saliva and tears. For this purpose, pilocarpine (Salagen) or cevimelin (Evoxac) are used.
- Use bromhexine or ACC to relieve dry upper respiratory tract in the development of sinusitis, rhinitis, laryngitis and / or bronchitis.
- Appointment of local lubricants to eliminate discomfort during sexual intercourse. In postmenopausal women, local and systemic estrogen-based drugs are prescribed.
When treating non-iron manifestations of the syndrome:
- Low doses of GCS or NSAIDs are prescribed with minor systemic symptoms.
- Small dosages of GCS are prescribed in combination with leukeran. Their use is advisable with a significant increase in the salivary glands (only with the exception of lymphoma), the absence of severe systemic disorders, the presence of moderate or serious deviations from the norm in laboratory indicators. Such medicines have been used for several years.
- Cyclophosphamide is administered for vasculitis.
- High doses of GCS and cytostatics are prescribed in combination with intensive therapy for severe systemic manifestations of the syndrome.
Intensive treatment is carried out with severe systemic disorders and the risk of serious, life-threatening complications in order to eliminate increased immuno-inflammatory activity, as well as to change the nature of the pathology and improve the general condition of the patient. Genetically engineered biological preparations are successfully used to control systemic extra-iron manifestations and reduce functional glandular insufficiency.
To date, there is no specific prevention of a disease or Sjogren syndrome. But you can prevent its recurrence, as well as reduce the intensity of symptoms due to:
- taking medication prescribed by a doctor;
- prevent the adherence of secondary infections;
- limit the effects on the body of factors that provoke exacerbation of symptoms;
- stress avoidance;
- daily humidification of indoor air;
- avoidance of exposure to the body.
Sjogren syndrome has life-friendly projections. Timely initiated therapy helps to slow the progression of the pathology and rehabilitation of the patient. With late treatment, the risk of developing dangerous complications arises and grows, which can later lead to disability or death of the patient with this syndrome.