Sjogren's syndrome, or "dry syndrome," manifests itself as a decrease in the function of the glands of external secretion, which causes a pronounced dryness of the skin, mucous membrane of the mouth, eyes, nasopharynx, trachea, vagina, as well as a decrease in the secretion of digestive enzymes by pancreatic cells and t
It more often accompanies a number of autoimmune diseases of connective tissue - scleroderma, dermatomyositis, etc. - and is called the secondary syndrome of Sjogren. In the case of an independent disease, it is called the primary syndrome or Sjogren's disease.
Epidemiological studies that have been conducted in recent years have made it possible to detect the presence of this pathology in 0.59 to 0.77% of the total population, among people over 50 years old, 2.7%.The incidence among women is 10 - 25 times higher than among men.
Causes and mechanism of the development of the disease
The most characteristic for the disease, Sjogren's syndrome are the lesions of the salivary and l
The causes of Sjogren's syndrome are not fully established. The most accepted is the theory of the pathological immune response of the body. It occurs in response to damage to the glandular cells by the retrovirus, in particular the human immunodeficiency virus, cytomegalovirus, VI herpes virus, Epstein-Barr virus. Despite the similarity of immunological disorders and changes in the body when the virus is infected, there is no direct evidence of a viral cause of the disease.
The viruses themselves, as well as the epithelial gland cells they alter, the immune system perceives as foreign elements( antigens).It produces antibodies on them, which attack and gradually destroy the glandular tissue. The disease is often found as a family, especially among twins, which gives rise to the assumption of a genetic predisposition.
Thus, it is assumed that the combination of many factors is important in the mechanism of the onset and development of the disease:
- Viral( possibly).
- Immune control by T-lymphocytes.
- Immune regulation with the participation of sex hormones, as evidenced by a rare incidence in childhood( up to 20 years);and among girls in 80% are sick girls.
- Stress response of the body resulting from an immune response.
Symptoms of Sjogren's syndrome
All symptoms of Sjogren's disease are grouped in 2 groups:
- Glandular - lesion of epithelial secreting glands, accompanied by a decrease in their function.
- Extrafat, or systemic - a variety of symptoms that indicate the defeat of various body systems and are not specific for the disease.
A permanent and mandatory symptom for the diagnosis of Sjogren's syndrome is the defeat of lacrimal and salivary glands. In the early stages of Sjogren's disease, the dryness of the mucous membranes is felt by the patient, usually only with excitement or physical exertion, as the disease progresses, the feeling of dryness becomes permanent, forcing the patient to drink food, often moisturize the mouth and use drops, an artificial tear to moisten the eyes.
Symptoms of keratoconjunctivitis are a feeling of scratching, sand and burning in the eyes, itching of the skin in the eyelids and their redness, periodic accumulation in the corners of the eyes of the separated white color. There is a decrease in visual acuity, narrowing of the eye gap, pinpoint hemorrhages and point infiltrates( edema) of the conjunctiva, increased sensitivity to bright light( photophobia) - unpleasant sensations, lacrimation, pain and pain in the eyes( see details of dry eye syndrome: treatment, symptoms).
The consequence of deep dryness of the cornea is its turbidity and the formation of corneal trophic( malnutrition) ulcers. Accession of staphylococcal infection causes purulent conjunctivitis, suppuration of corneal ulcers followed by a terrible complication in the form of its perforation( perforation).Sometimes there is an increase in the lacrimal glands themselves.
Chronic parenchymal parotitis
It is the second permanent and mandatory sign of Sjögren's syndrome and is characterized by a common lesion of the salivary gland tissue. Very often this is preceded by stomatitis, reddening and dryness in the fringe and corners of the lips "jamming", caries of many teeth, enlargement of submandibular and cervical lymph nodes.
Further dry mouth and increased parotid salivary glands, much less often - palatine, sublingual and submaxillary glands. Dryness in the oral cavity occurs initially with insignificant psychoemotional or physical exertion, but afterwards it becomes permanent and leads to the need for moisturizing in the mouth during conversation and eating, especially when eating dry foods.
The disease occurs in 50% of patients with exacerbations, during which the parotid glands are enlarged, leading to a change in the contours of the face( "hamster's face").The glands are slightly painful at palpation or generally painless. In the period of remission, they decrease, but after one of the next exacerbations remain increased. Approximately 30% of patients have glandular enlargement not only during relapses, but gradually and continuously.
The mucous membrane of the tongue and mouth become dry and red( lacquered), easily traumatized and sometimes bleed, and saliva - poor foamy or viscous.
In the absence of treatment in the future:
- described symptoms become more pronounced
- papillae of the tongue are smoothed and atrophied, wrinkles appear on it, glossitis arises( inflammation of the root of the tongue), which makes it very difficult to swallow
- on the mucous cheeks, parts of the cornified epithelium appear, andsaliva completely absent
- possible attachment of a bacterial, fungal or viral infection
- on the lips there are cracks and crusts
- partially or completely drop out teeth
On the above groupsSymptoms are based on the diagnosis of Sjogren's syndrome, while the remaining signs may accompany the disease in certain variants, but are not decisive in the diagnosis.
Concomitant signs of lesions of the
- glands Dryness of the upper respiratory tract mucosa
This leads to hoarseness of the voice, chronic rhinitis and inflammation of the mucosa of the paranasal sinuses, otitis and hearing loss. Atrophy, dryness, redness and swelling of the vaginal mucosa are the cause of chronic colpitis, accompanied by burning, pain, itching, decreased sexual desire.
- Frequent symptoms are dry skin, reducing sweating.
In 30% of patients, there is a lesion of sweat apocrine glands located in the armpits, lower abdominal wall, in the pubic region and external genitalia. This contributes to skin peeling, pigmentation in these areas and the formation of abscesses and phlegmon upon attachment of a secondary infection.
- Symptoms of digestive system diseases( in 80%)
These are diseases such as chronic esophagitis, gastritis, chronic cholecystopancreatitis( see pancreatitis: symptoms, treatment, what can be eaten with pancreatitis).They are manifested by eructations, pain behind the breastbone when food passes through the esophagus, pain in the epigastric region and the right hypochondrium, nausea and sometimes vomiting. With a decrease in the secretion of digestive enzymes, pancreatic cells appear to be intolerant to fatty and dairy products, as well as a clinical picture of intestinal dysfunction.
Overweight symptoms of
These include bone pain without radiologic changes in them. In 60% of patients, stiffness, pain and restriction of movements in small joints are noted mainly in the mornings, less often in large joints.5-10% have pain and minor weakness in the muscles, sometimes severe polymyositis( a common inflammation of the muscle groups) can develop.
In 50% of cases of Sjogren's syndrome, tracheobronchitis occurs, accompanied by coughing and shortness of breath, and at an x-ray examination in 65% of patients chronic interstitial pneumonia or pulmonary fibrosis is detected, much less exudative( exudate) and dry pleurisy( see pleurisy: symptoms, treatment).
The majority of patients show an increase in the occipital, submandibular, cervical and supraclavicular lymph nodes, in 30-35% lymphadenopathy( enlarged lymph nodes) is generalized in nature. In this case, an increase in the spleen and liver is often observed.
The same percentage suffers from vasculitis( inflammation of the inner shell of the arteries), which proceed according to the type of erased forms of Raynaud's syndrome or obliterating atherosclerosis of the lower limbs. The defeat of vessels of small caliber is manifested in the form of various skin small-spot or spotted skin rashes, accompanied by itching, burning and rising temperature, the formation of ulcers and necrotic( necrosis) areas of the skin.
The widespread defeat of the peripheral nervous system( polyneuropathy) is clinically manifested by the disorder or loss of skin sensation on the hands and feet by the type of "gloves" and "socks", less often neuritis of the facial nerve, trigeminal nerve, and damage to the vessels of the membranes of the brain and spinal cord.
The examination also allows to establish signs of thyroid damage with a decrease in its function( about 10%), a tendency to allergic reactions to many foods, medications and household chemicals.
Diagnosis of "Sjogren's disease" is established on the basis of the presence of two main diagnostic criteria - keratoconjunctivitis and parenchymal parotitis, but only after exclusion of the third criterion - systemic autoimmune diseases( systemic lupus erythematosus, rheumatoid polyarthritis, etc.).If there is a third criterion, the diagnosis of Sjogren's syndrome is established.
Laboratory testing methods are optional. They allow for differential diagnosis and to some extent to judge the degree of activity of the process:
- high ESR
- anemia, decrease in blood levels of leukocytes and platelets
- increase in blood levels of immunoglobulins A, G and M
- presence of rheumatoid factor
- presence of antibodies to components of cell nuclei, determined immunofluorescent method.
The Schirmer test is also used, which allows to determine the level of decrease in tear release after stimulation with ammonia, staining of the cornea and conjunctiva with special ophthalmic dyes followed by biomicroscopic examination of the epithelium.
To investigate the function of the salivary glands, the following methods are used:
- method of unstimulated and stimulated sialometry - the amount of saliva released per unit time
- sialography - is performed by X-ray method after introduction of contrast agent into the ducts of the salivary glands;the technique makes it possible to identify in the gland cavities over 1 mm
- microscopic examination of salivary gland biopsy
Treatment of Sjogren's syndrome
Treatment of Sjogren's syndrome is performed depending on the stage of the disease and the presence of systemic manifestations.
In order to stimulate the function of the glands, the following is carried out:
- drip injection of the contralateral
- subcutaneous injection of galantamine
- as an symptomatic treatment, "artificial tears"( droplets in the eyes) with low viscosity Lacrisifi( 200-250 rub), natural tear( 250 rubles)medium viscosity Lakrissin, high viscosity Ophtagel 180 rub, Vidisik 200 rub, Lakropos 150 rub.
- with a fortifying purpose is conducted courses of vitamin therapy
At the initial stages, in the absence of damage to other systems and unexpressed laboratory changes, long courses of glucocorticosteroids( prednisolone, dexamethasone) in small doses are prescribed.
If the symptoms and laboratory indicators are significant, but there are no systemic manifestations, cytostatic immunosuppressive drugs - cyclophosphamide, chlorbutin, azathioprine - are added to corticosteroids. Supportive therapy is carried out by the same means for several years.
In the presence of symptoms of systemic damage, regardless of the stage of the disease, corticosteroids and immunosuppressants are immediately prescribed in high doses for several days with a gradual transfer to maintenance doses.
In generalized polyneuritis, vasculitis, renal damage and other severe manifestations of the disease, such methods are added to the above treatment, extracorporeal treatment - plasmapheresis, hemosorption, plasma ultrafiltration.
Other drugs are prescribed depending on complications and concomitant diseases - cholecystitis( see what can be eaten with cholecystitis), gastritis, pneumonia, endocervicitis, etc.
In certain cases it is necessary to follow dietary intake and restrict exercise.
Sjögren's syndrome is not threatening for the life of the patient, but can reduce the quality of life and lead to disability. Timely diagnosis and treatment in the early stages of the disease can significantly slow down the process of development of the pathological process, prevent serious complications and retain work capacity.