SHEGREEN SYNDROM - autoimmune inflammatory pathology manifested lesions of exocryne glands - tear, salivary, sebaceous, sweat, digestive. The syndrome was first described in the late 19th century with an ophthalmologist from Sweden H. Shegran, in honor of which he got his name. Shegran observed on patients who complained about dryness in the eyes and mouth, as well as pain in the joints. After some time, scientists of related medical regions were interested in this ailment.
Factors contributing to the development of pathology - heredity and autoimmune response to viral infection. SHEGREEN syndrome develops with a decrease in immunity. Immune system perceives own cells The body as foreign and begins to produce antibodies against them. Immunoagressive reactions are accompanied by lymphoplasmatic infiltration of exocryne glands. Inflammation applies to all organs and fabrics of the patient. With damage to the glazed gland glands, their dysfunction occurs, and the operation of the secret is reduced. This is how the development of "dry syndrome" occurs. The hypofunction of exocryne glands is usually combined with systemic immunipality diseases: periatery, vasculitis, dermatomyositis. The generalized form of pathology is characterized by the defeat of the kidneys with the development of an interstitial aseptic nephritis, the vascular vessels with the development of vasculitis, lungs with the development of pneumonia.
Pathology develops mainly in women of menopausal age, belongs to the most common autoimmune pathologies and requires comprehensive therapy. The syndrome has a code on the ICD-10 M35.0 and the name "dry SHEGREEN Syndrome".
SHEGREEN SYNDROM is manifested by dry mouth, burning in the vagina, sharp in the eyes, in the throat. Elegant manifestations of pathology - arthralgia, myalgia, lymphadenitis, polynevrit. The diagnosis of the syndrome is based on clinical data, the results of laboratory studies and functional tests. Timely treatment of pathology glucocorticosteroids and cytostatics makes a favorable disease forecast.
Forms of pathology:
The degree of activity of the syndrome:
The etiopathogenetic factors of SHEGREEN syndrome are currently unknown. This syndrome belongs to autoimmune diseases. Perennial research of scientists was established that the ailment develops under the influence of negative factors on the human body predisposed to the syndrome.
Factors promoting the development of syndrome:
Pathogenetic syndrome links:
Lymphoid infiltrates occur not only in exocryne glands, but also in the internal organs, joints, muscles, leading to their dysfunction and the appearance of appropriate symptoms. Gradually, the foci of inflammation lose their benign character, they acquire polymorphism and deep into the surrounding tissues are beginning to spread.
SHEGREEN SYNDROM - severe pathology, requiring immediate conduct of therapeutic and diagnostic procedures.
Irony symptoms are due to the damage to the epithelocytes of the secretioning glands, which is accompanied by their dysfunction.
Pathology is manifested by the following clinical signs:
Elegestic manifestations of pathology indicate the defeat of the internal organs and are not specific for this syndrome:
With absence timely diagnosis and rational therapy in patients develop severe complications and negative consequences.
The causes of the death of patients are:
The diagnosis of Shegren syndrome begins with the identification of the main clinical signs of pathology. Experts find out complaints of patients, collect history of life and illness, conduct an objective examination. CONCLUSION ON NEVH Specialists are made after receiving the results of additional research methods:
Main diagnostic techniques:
Timely diagnosis and earlier treatment will help to cope with this ailment. Otherwise, the risk of developing severe complications and a deadly outcome is significantly increasing.
Specific treatment of SHEGREEN syndrome is currently not developed. Patients conduct symptomatic and supporting therapy.
In the launched cases, extracorporeal treatment is carried out - plasmapheresis, hemosorption, plasma ultrafiltration.
Lack of timely I. proper treatment It may cause the disability of patients. Adequate therapy, appointed by a rheumatologist, stops the further development of pathology, prevents severe complications and maintains ability to work.
Specific prevention of SHEGREEN disease is not developed. Preventive measures to prevent the aggravation and progression of the disease:
SHEGREEN SYNDROM - chronic pathology With frequent change of exacerbation and remission periods. Due to the constant decline of forces, muscle weakness and articular pain, the quality of life of patients is reduced. Acute pneumonia, renal failure or oncopathology - frequent reasons The death of patients. The combination of SHEGREEN syndrome with another autoimmune disease significantly worsens the forecast for recovery.
SHEGREEN DISHING TREATMENT MUST be complex and directed both to suppress immune disorders in the body and on clinically manifested lesions separate organs and systems. The leading role in the treatment belongs to a rheumatologist who performs comprehensive therapy Together with the oculist, dentist, and if necessary, with other specialists.
As prophylactic measures applied dispensary observation At the rheumatologist with attracting for joint therapy of the dentist, the oculist, the gynecologist and the constant reception of supporting doses of drugs, small doses of hormones (5 mg), cytostatics (chlorubutin, cyclophosphane) and coursework are necessary preventive treatment symptomatic means In order to achieve the state of clinical remission.
It is important to rehabilitate chronic infection ( chronic tonsillitis, sinusitis, carious teeth, stomatitis). It is recommended to avoid hypoints, cold illness and neuropsychic overvoltage. The primary prevention of SHEGREEN disease is not designed.
During the period of exacerbation of the disease, restriction of speech loads is recommended due to xerostomy (dryness in the mouth). To reduce the light-wide distribution in patients primary syndrome Shegren, We recommend wearing points with darkened glasses (in bright sunny days).
Drug therapy for SHEGREEN Disease is based on corticosteroid hormones and cytostatics.
Large therapy of dental and ophthalmological manifestations of SEGREEN disease is of great importance. As a result of a decrease in the amount of saliva and its protective properties of the manifestation of xerostomy, most patients are exacerbated by the accompanying candidiasis of the oral cavity. To combat candidiasis, the irrigation of the oral cavity is used by a 2% reduction soda solution, nastatinoma lubrication, nystatin is assigned to 500,000 cells 4-6 times a day (or left-choice, diflkzhan). Pills antifungal antibiotics No need to swallow, but should be dissolved in the mouth. In the occurrence of herpetic lesion, the mucous membrane uses oxolin or methyluracil oxic oxisol, acyclovir.
For the treatment of parenchymal vapor, compresses from 30% of the dimexide solution can be used. They are superimposed daily to the area of \u200b\u200bthe near-dry and submandibular salivary glands for 30-40 minutes.
The timely treatment of carious teeth and the prosthetics of the oral cavity is essential.
Local therapy of ophthalmological manifestations of SHEGREEN disease is aimed at overcoming hypolacrymy, improving the regeneration of the cornea epithelium and conjunctiva, prevention of secondary infection. A 0.5% methylcellulose solution is used as artificial tears. It is buried from 3 to 12 times a day, depending on the severity of lesions. To combat infection, solutions of albucide, leftomycetin, furaticiline are used.
In case of SEGREEN disease, the atropine-containing and antihistamines capable of reduce the secretory function of the exocrine glands are contraindicated.
Non-media treatment. Medical diet.
A physiologically full-fledged diet with a sufficient amount of vitamins is appointed. It is recommended 5-6 meals a day. The use of chewing gum is shown.
Sanatorium-resort treatment during the diseases of the Shegon-Gastrointestinal Profile.
Content
it an autoimmune disease It is concerned relatively often, the connecting tissues amazes. Diagnosed in most more often in women at middle age. The development of Shengren's disease is 30%, if the patient has the following autoimmune diseases: sclerodermia, SLE, RA, mixed illness connective tissue, vasculitis, primary biliary cirrhosis, Tareloit Hashimoto, autoimmune hepatitis.
The disease is characterized by an autoimmune systemic lesion of the connective tissue, which is accompanied by elegant or ferrous manifestations. The typical manifestations of pathology include a decrease in the function of secretion of salivary and the lacrimal glands, which leads to the development of dryness in the nasopharynx, the burning in the eyes. The following extra-zealous manifestations of the disease are distinguished:
As a rule, the course of illness is benign. According to statistics, this disease is leading among collagenosis, much more often diagnosed in women aged 20-60 years. In men or in childhood There is a pathology less often. The exact reasons for the development of pathology are not established, the predetermining factors often seruate an autoimmune response to a viral infection or hereditary factor.
The disease provokes an immunoagressive reaction of the body, which leads to the appearance of antibodies to its own tissues, lymphoplasmic infiltration of gonducts of glands (tear, gastrointestinal tract). In generalized form, 30% of patients develop kidney damage (abacterial jade), muscles (myiositis), lungs (interstitial pneumonia, vessels (productive vasculitis)). Sex primary and secondary SHEGREEN syndrome. Against the background of pathology, arthritis of similar on the grounds with rheumatoid, the defeat of the exocrine glands may appear. Code of the ICD-10 - M35.0 dry SHEGREEN syndrome.
The factors causing the development of pathology are not fully investigated, so doctors are based on perennial information. It is noted that the syndrome is usually due to the factors of the external environment, negatively affecting the patient's body, which has a predisposition to pathology. The disease passes into the active phase when the immune system is activated. The main reason is the violation of the regulation of B-lymphocytes in the blood, the presence of hypersensitivity.
Degenerative processes, necrosis, atrophy of the acinar glands, decrease in tear and salivary secrets due to the lesion of the exocrine glands. Against this background, the pathology of nerve fibers is developing, which leads to drying and dry cavities. The reason for the development of SHEGREEN syndrome can act:
The main reason why autoimmune processes are developed - the absence of compensation for the main needs of the body. The genetic factor is partly legitimate, because there is a function of genes to coding, determining the reaction rate under concrete conditions. It becomes the cause of the predisposition of the descendants to the disease, if it was at their ancestors.
In men, Shegren syndrome happens only in 5-10% of cases, the rest fall to women. This is due to the hormonal background during menopause. The defeat of the body can develop under the action of hepatitis, different viruses, rotavirus and herpes. These reasons are not proven scientifically, therefore, their accuracy is in doubt. The disease belongs to the group of heavy pathologies, therefore requires timely diagnosis, immediate treatment. In addition to glands, SHEGREEN syndrome can cause the defeat of the following organs:
SHEGREEN SYNDROM is divided by the advent of the patient's appearance, therefore, in medical practice, the following two main forms are distinguished:
In medical practice, manifestations are divided into extra-zeal and fermented. The latter are due to the defeat of the secreting glands, which is manifested in reducing their function. The following main symptoms of the disease are distinguished:
Elegest symptoms of SHEGREEN syndrome are diverse, are systemic. Among the common manifestations are allocated as follows:
When conducting laboratory studies to determine the SHEGREEN syndrome, a general blood test is used, which should show anemia, moderate leukopenia, acceleration of ESO. If you have a biochemistry, it will show the growth of the level of U-globulins, fibrin, a common protein, serumcoid, sialic acids, the content of cryoglobulins will be found. When conducting an immunological reaction, it will be revealed:
A broader test is performed, in which a specific reaction is marked. Displays the analysis in response to the stimulation of the ammonic alcohol, the reduction of tear products. If there is a marking of conjunctiva, cornea with dyes, then dystrophic foci of epithelium, erosion will be revealed. To confirm the diagnosis, the following tests are prescribed:
The course of therapy is appointed only by the attending physician, self-medication is prohibited. Groups of medicines and specific drugs are selected depending on the stage of the disease, accompanying pathologies, symptoms. In the treatment, follow the following principles:
For symptomatic treatment with eye damage, local therapy is used to eliminate dryness, protection against the addition of secondary infection. With dryness, the use of artificial tears is prescribed, depending on the degree of damage to the organ eat a few drops from 3 to 10 times a day. To protect the cornea, soft healing contact lenses are needed. For the prevention of infection penetration, a solution of furaciline, ciprofloxacin, 0.25% solution of Levomycetin is prescribed.
With the inflammation of the chronic salivary glands, it is necessary to direct treatment for strengthening the walls of the ducts, overcoming dryness, improving the recovery of the epithelium of the mucous membrane, the prevention of exacerbations, the fight against secondary infection. For the normalization of secretion, the power of the salivary glands is used by novocaine blocks. If the aggravation of chronic vapotitis began, it is necessary to apply an applique of 10-30% solution of dimexide. For the treatment of purulent vapotitis, the following procedures are carried out:
To accelerate the healing of the red border of the lips, the mucous membrane of the mouth when cracking, erosions can use sea buckthorn butter, rosehip, solcelovoy, methyluracyl ointment. Decamin caramel will help, processing the ENKAD mucosa, active derivatives of nucleic acids. With dryness of the nasal mucosa, the frequent appliques of isotonic sodium chloride solution are prescribed.
With bronchial dryness, trachea prescribe long-term use of bromgexine (3 times a day 8-16 mg for 3 months). Therapy of symptoms of chronic gastritis with secretory insufficiency is carried out with replacement therapy: pepsidyl, natural gastric juice, hydrochloric acid. Treatment of pancreatic failure is carried out using enzymes:
Depending on the manifestations of pathology, you need to adjust the diet (diet), take medicines. Funds folk Medicine Aimed only to eliminate concomitant symptoms and do not act as self-therapy. Overcome signs of malaise with the following tools and rules:
Shegren's syndrome does not bear a threat to life, but significantly reduces its quality and comfort. You can avoid complications with timely diagnosis, the beginning of treatment. This will help slow down the progression of the disease, keep the patient's ability to work. In the absence or ineffectiveness of therapy, the consequences are developing, which lead to the disability of the patient. The main complications in the SHEGREEN syndrome include:
Shegren syndrome has a favorable forecast for life. Timely disease therapy helps to slow down the progression of pathology, which helps the patient to lead a full-fledged life. If the disease is launched, it can lead to person's disability, but without fatal outcome.
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Syndrome or SHEGREEN symptom ("dry eye") is the pathological condition of the organism, in which the functioning of the gland of the external secretion is reduced, the dryness of the skin, mucous membranes increases, the secretion of digestive enzymes produced by the pancreas is reduced.
The disease is assigned the code on the ICD-10 - M35.0 - dry syndrome (SHEGREEN).
The syndrome is divided into primary when it occurs independently, regardless of other concomitant diseases, and secondary, when it develops against the background of another autoimmune pathology of the connective tissue. What methods of the treatment of the disease suggests today medicine, as well as methods of prevention of syndrome Consider more in the article.
What is the reason for the occurrence of the syndrome, it is definitely impossible to say. Some researchers believe that the disease is manifested as a result of an atypical immune system reaction on certain pathogenic microorganisms. Among those are the Epstein-Barr virus, herpes virus, cytomegalovirus, HIV, which can damage the cells of the external glands. Nevertheless, this reason for the development of the disease is not yet fully studied, and the direct evidence of the role of these viruses in the formation of the syndrome was not found.
Vital activity in the body of the virus that can become a source of changes in the epithelial cells of the external glands, which are considered immune systemlike alien agents. The body gives an immune response, actively producing antibodies against altered cells and viruses, causing the destruction of the first.
An autoimmune type of syndrome formation is not the only assumption of scientists. There is another theory of occurrence - hereditary. Special studies were conducted, on which it was established that the syndrome often arises from individuals whose close relatives suffered from this pathology.
If a woman suffers from Shegren syndrome, the risk of its development increases and has a future child
So, such factors can affect the manifestation and development of syndrome:
This body disorder specialists are divided into several species. Given the reason for the development of pathology, there are the following forms:
There is a different classification of SHEGREEN pathology, given the nature of the development and features of further exacerbation:
Symptoms of the disease of the SHEGREEN is quite diverse. It is necessary to know which symptoms arise first in order to consult a doctor in time and prevent dangerous consequences pathology.
Initial clinical manifestations Presented in the form:
Over time, other symptoms are joined:
Another sign of the development of the disease is an increase in lymph nodes. In particular, it concerns the parole lymph nodes.
The lower facial part increases, that is, the chin. The dry tongue is covered with shallow rash, which over time goes into minor ulcers.
The traditional method for determining the presence of saliva in the mouth: put on the tongue of a small piece of lemon. If saliva does not occur, it can indirectly indicate the development of the disease of the SHEGREEN.
Drag and lips on which ulcers and blisters begin to form. In the risen nasophal cafe appears crust. With the development of the syndrome, frequent nose bleeding occurs. After 2-3 weeks after the start of the active pathological process, the voice completely disappears.
Penetrating in the body viral infection It can involve not only external glands in the defeat, but also any internal organ.
Based on the state of the kidneys and organs of the digestive tract, you can consider the picture of the appearance of pathology. In the SHEGREEN syndrome, cirrhosis of the liver is often developing or deformation of the organ cells, which subsequently generates hepatitis. The main symptoms in this case are nausea and vomiting, the taste of bitterness in the oral cavity. Due to the painfulness of food intake, the appetite worsens, and also increases the risk of ulcer's development due to the fact that the acidity of the stomach is reduced.
The genitals are also affected by women. Such a phenomenon has a special danger. It occurs itching syndrome, burning and drying the mucous membranes into the vagina. Due to the absence of moisturizing in this area, the process of sexual contact is disturbed, since the lubricant does not occur. However, it is impossible to say for sure that the woman develops precisely, it is impossible, because such symptoms may occur with other gynecological diseases (for example, with vaginine).
It is worth noting that the SHEGREEN syndrome is reflected in the skin: decreased or completely stops the sweating, the dryness of the epidermis increases, the stoves with peel appear on it, the sebaceous glands occurs. Over time, ulcers are formed on the skin, which are moving to the gloomy.
The functioning of the muscular and the articular apparatus is disturbed, it becomes difficult for a person to blend and bend limbs. A person is concerned about the constant weakness of the whole torso and the general decline of forces.
Muscles hurt even at rest.
In some cases, an oncological disease is developing, for example, a malignant tumor is formed at the site of drying the glands. If you do not start timely treatment of the syndrome, there is a significant failure in the work of immunity, which may cause for the development of skin cancer, and sometimes - fatal outcome.
All the symptoms of the diseases of the Shegren can be divided into the stages of its development:
At the initial stage of the flow of the syndrome there is dryness in the oral cavity and on the lips, stomatitis and caries appear. According to the results of laboratory studies, it is possible to identify signs of a weak inflammatory process.
Over time, the person begins to bother the light-friendly, the acuity of sight is reduced, the eye slots are narrowed. The second mandatory sign of the syndrome is an increase in the size of the salivary glands. At the stage of the stage, the increase in dryness of the mucous meal occurs only during exercise, and at 2 stages - on an ongoing basis. There is almost complete absence of saliva in the mouth.
In the late stage of the main clinical sign Diseases - complete absence of salivation. This is the cause of swallowing, speech.
Put the correct diagnosis is not so difficult. Today, medicine has various diagnostic techniques that allow you to determine developing disease. The most successful method is laboratory tests. For SHEGREEN syndrome, it is characterized by an increase in the erythrocyte sedimentation rate (EE), a decrease in blood leukocyte content, hypergammaglobulinemia, antinuclear rheumatoid factor in stock.
Another diagnosis criterion is an external inspection. The doctor draws attention to the following signs:
If such symptoms taken separately are not considered specific precisely for SHEGREEN syndrome, then the presence of 4 of those in 80% of cases indicates that this pathology.
Lab diagnostics will help confirm the diagnosis.
An important diagnostic condition is a differential analysis. SHEGREEN syndrome differentiate with such diseases as rheumatoid arthritis, systemic red lupus, autoimmune diseases of the biliary tract and kidneys.
It is possible to treat the disease only after the appropriate diagnosis was carried out and the final diagnosis was made. To completely get rid of the syndrome, unfortunately, it is impossible. For this reason, only symptomatic therapy is carried out. Recovery consists only in achieving long-term remission.
Though to fully cure the disease can not be able to direct all the forces to relieve general status With exacerbation. To do this, you can adhere to some simple recommendations, eliminating from life:
Treatment of SHEGREEN Symptoms is carried out using the following medicinal preparations:
Healing the symptoms of the disease can be in a complex with wearing glasses or contact lensesthat will help keep cornea moisture
Also requires control of the condition of the salivary glands. Dryness of the mucous mouth can cause not only unpleasant feeling, but also influence professional activities a person, for example, if it is connected with the need for a permanent conversation (TV presenter, lecturer, etc.). It is possible to normalize the functioning of the salivary glands with the help of special drugs that the doctor discharges individually.
Pregnancy - such a period in the life of a woman, during which not all medicines are allowed to use. In this case, may suffer future child: Forbidden drugs negatively affect the formation of organs and fetal systems. As a rule, under the SHEGREEN syndrome, a soft therapy of local exposure is carried out, which will help heal the symptoms of SHEGREEN syndrome:
In some cases, the patient is carried out with hemocorrection (filtering and blood transfusion).
At home, you can engage in self-medication, but the main thing is to no longer harm the already weakened organism. In any case, it is worth contacting a specialist who will tell you how to properly treat at home, and what is not recommended.
Often use the following folk remedies:
Any people's method Therapy should be agreed with your doctor.
Forecast for people patients with SHEGREEN syndrome, relatively favorable. This concerns those cases when therapy has begun in a timely manner, that is, at the initial stage of the development of the disease. Otherwise, hazardous consequences are not excluded, which include:
One of the complications of SHEGREEN syndrome is vasculitis (inflammation of the joints).
Late diagnosis (last stage) denotes that no therapy methods will bring recovery, and the person is waiting for a fast fatal outcome.
To date, there is no specific prophylaxis of SHEGREEN syndrome. The only one can prevent frequent exacerbation of the disease and slow down its progression in the following ways:
Shegren syndrome is characterized by frequent change of remission and exacerbation periods. Regular decay of forces, muscle weakness, pain in the joints - all this reduces the quality of life of a person, a patient chronic disease. High mortality is observed in cases where the disease is complemented by acute pneumonia, renal failure, oncopathology. Schogen syndrome is treated with experts quite successfully, if you do not miss the first alarming symptoms.
Schogrena syndrome (SSh) - a systemic autoimmune disease characterized by the development of autoimmune exocrinopathy, which leads to the occurrence of xerostomy and xerophthalmia, dryness of the mucous membranes of the nose, larynx, trachea, bronchi, female genital organs, atrophic gastritis. Statistical data. Prevalence: 0.6-3.0% in the overall population. The predominant age is over 30 years old. The prevailing floor is female (10-25: 1).
Code of PO international Classification MKB-10 diseases:
Etiology and genetic features. One of the etiological factors consider chronic viral infection (CMV, Epstaina Barr virus, retroviral HTLV - 1, HCV). In patients with HIV - infection develops lymphocytic sialdenitis, lymphocytic interstitial pneumonia resembling SS. Recove the association with HLA B8, HLA DW3, HLA DR3, HLA DRW52, allele DQA1 * 0501.
Pathogenesis. Chronic viral infection induces the activation of T - and B - lymphocytes. During the activation of CD4 + - lymphocytes, cytokines are synthesized (IL - 1, IL - 6, G - IFN, TNF - A), which induce the expression of the AG main complex of class II histocompatibility at the epithelial cells of salivary and peeling glasses. This leads to ag-specific activation of lymphocytes and the development of autoimmune reactions against exocryne glasses. Activated B lymphocytes synthesize G - globulins in large quantities. The activation of the in-lymphocytes passes three stages: polyclonal (clinically manifested by exocrinopathy), poly - oligo - monoclonal (detect clinical systemic manifestations), monoclonal (clinically detect lymphoma).
Classification. The primary CSH is distinguished, not associated with other rheumatic diseases, and secondary SS, arising against the background of rheumatoid arthritis, SLE, polyimosit / dermatomyositis, chronic active hepatitis, biliary cirrhosis, sarcoidosis and AIDS.
Clinical picture
The lesion of exocryne glasses .. Dry keratoconjunctivitis: itching, burning, rubbed in the eyes, when the infection is joined - ulcers and perforations of the cornea .. Sialadenit: dry mouth, chronic parenchymal paotitis, in the later stages - progressive progressive caries, difficulties when swallowing food .. Chronic pancreatitis.. dryness of the mucous membranes of the nose, pharynx, larynx, trachea, genital organs, skin .. lesion gastrointestinal tract: chronic atrophic gastritis with secretory insufficiency, damage to the duodenum with the occurrence of hypochlorine dyskinesia, damage to the colon with the appearance of pain in its palpation and the development of constipation patient.
Systemic manifestations .. The defeat of the joints: arthralgia, recurring non-erosive arthritis of small joints with the development of deformations (ankylose) .. The defeat of the muscles: Malgia, Miositis .. The lesion of the lymphatic system: regional lymphadenopathy, generalized lymphadenopathy, lymphoprolifemia disease (lymphoma, macrooglobulinemia Valdenstram) . Clinical development The latter can be suspected with a rack and significant increase in varnish salivary glands, generalized lymphadenopathy and splenomegaly .. Light damage: interstitial pneumonia, interstitial light fibrosis, pleurisy .. Kidney damage: kidney acidosis, aminoacidurium and phosphatia with nephrogenic diabetes development, chronic interstitial nephritis (glomerulonephritis It rarely occurs, more often in patients with cryoglobulinemia), amyloidosis .. Rayno phenomenon .. Vasculitis (5-10%) .. leather skin: hypergammaglobulinemic or cryoglobulinemic purple, chronic ulcers, skin ... defeat nervous system: Multiple Monoeurite, aseptic meningitis, epileptic seizures, CNS lesions, resembling multiple sclerosis.. thyroiditis with hypo - or hyperfunction of the thyroid gland.
Features of HIV - associated SS. Frequent defeat of men. Association with HLA - DR5. Low frequency of xerofthalmia. Lymphocyte hepatitis, gastritis, nephritis, interstitial pneumonia, aseptic meningitis.
Laboratory data
OAK: Increased ESP, leukopenia. Possible hemolytic anemia. Thrombocytopenia is not characteristic; When it is discovered, it should be done bone marrow To eliminate myelodsplastic reactions, lymphoma, medicinal myelodepresses. Hypergammaglobulinemia. Mixed monoclonal cryoglobulinemia type II. In the study of the anat by indirect immunofluorescence, the peripheral (corresponding to the presence of AT to DNA) is detected (corresponding to the presence of AT to ribonucleoproteins) types of luminescence. With the help of immuno-immunimen methods, AT to RO - La is detected and to organic-specific agricultural agents of stomach, thyroid gland, smooth muscles. Availability of the Russian Federation (95%) in high titer. An increase in the concentration of CRH is not typical. OAM: Proteinuria (rarely). Detection in the urine of lung chains of monoclonal immunoglobulins (with SS with system manifestations).
Tool data
Dry keratoconjunctivitis .. Swirder test: reduced tears (less than 10 mm in 5 minutes). The test makes it possible to estimate the amount of tears allocated for 5 minutes after irritation by the strip of the clock paper, laid down for the lower eyelid. The indicator is the length of the strip, moistened with tears (norm - 15 mm in young, 10 mm in the elderly) .. Coloring the epithelium of the conjunctiva and cornea by Bengali pink and fluorescene. Test using the Bengal pink dye highly specific and informative. With SS, Bengali pink stains point erosion conjunctiva and sections of the spontaneous epithelium.
Parenchimato vapotitis .. Syalography: cavity detection with a diameter of at least 1 mm. Reducing the secretory activity of the salivary gland during stimulation of ascorbic acid - less than 2.5 ml in 5 minutes. Biopsy of salivary glands of the bottom lip: lymphoplasmascular infiltration (more than 1 focus consisting of more than 50 lymphocytes).
Diagnostic tactics (Vitali C. et al., 1993)
Ophthalmic symptoms .. dry eyes for the last 3 months or more .. The feeling of sand in the eyes .. Using the drug is an artificial tear of more than 3 p / day.
Dental symptoms .. Feeling dryness in the mouth over the past 3 months or more .. The recurrent or persistent healing of salivary glasses in adulthood .. the need for dry food with liquid.
Ophthalmological manifestations. Objective signs of eye lesion mounted on the basis of one of 2 tests .. Test Wirerah: Moisturizing paper strips less than 5 mm for 5 minutes .. account when painting Bengali pink\u003e 4 BijsterVeld system.
Histopathological signs: In the bioptate of small salivary glands, more than 1 conglomerate of mononuclear cells in the iron tissue with a volume of 4 mm3 are determined.
Objective signs of lesions of salivary glands mounted using scintigraphy, sialography or sialometry (nesomalized saliva current is<1,5 мм в течение 15 мин).
Autanthelia. The presence of at least one of the following types of AT. AT to RO / SS - A or LA / SS - B .. Anat .. RF.
The diagnosis is considered to be reliable in the presence of 4 out of 6 above the listed criteria. Sensitivity - 93.5%, specificity - 94%.
Exception criteria Diagnosis SS: preexisting lymphoma, AIDS, Sarcoidosis, "transplant against host reaction".
Differential diagnosis. The identification of the clinical picture of the SS sets the question primary this syndrome or secondary. It should be remembered that in addition to systemic diseases of the connective tissue, the SS can develop within sarcoidosis or AIDS. It is important to search for HIV. During sarcoidosis on the radiograph of lungs - the characteristic changes in the binding lymph nodes.
TREATMENT
Total tactics. The combination of systemic administration of immunosuppressive preparations and local replacement therapy.
Drug treatment. The initial stage of the disease in the absence of signs of systemic lesion .. prednisone 10-15 mg / day with a gradual decline in the dose 2-3 weeks to supporting 5-10 mg / day .. hydroxychloroquing 0.4 g / day, chloroquine 0.25 g / SUT. With pronounced manifestations of lesions of exocrynetic glasses in the absence of systemic manifestations .. Prednisolone 10-15 mg / day, then the transition to a supporting dose of 5-10 mg / day. Chlorambucil 4-6 mg / day with a transition to a supporting dose 2-4 mg / SUT. Systemic manifestations (lung fibrosis, cryoglobulousmic vasculitis) .. pulse - therapy with methylprednisolone and cyclophosphamide (for detail, for details, a systemic red lupus), then long-term therapy with prednisone and chlorambucal. In the treatment of cytostatics, it is necessary to regularly conduct common blood tests, urine, liver functional samples. With concomitant HIV - cytostatic infections are contraindicated .. during cryoglobulinemia - plasmapheresis. Ophthalmological manifestations .. Locally 0.5% P - P methylcellulose .. Therapeutic contact lenses .. For the prevention of secondary infection - washing the eye of the eye of nitrofuras, citrals with riboflavin. Dental manifestations .. Novocaine blockages of near-dry saliva glasses .. Introduction to the ducts of salivary glands of antibiotics .. Appliques of 30% R - Ra Dimethyl sulfoxide to the area of \u200b\u200bthe near-dry and submandibular salivary glands - 20 sessions of 25-30 minutes.
Complications. Lymphoma. The ulceration of the cornea. Ulcers of limbs when developing vasculitis.
Forecast. Life forecast is generally favorable. The forecast is deteriorating in the development of mellow fibrosis, cryoglobulinemia and, especially, lymphoproliferative diseases.
Synonyms . Dry syndrome. Kerodermatosis. The forerunner of the forerunner-grandfather. Syndrome Geuzheo-Schogrena.
Reduction. School - Schogren syndrome.
MKB-10. M35.0 dry syndrome [Schogrena].