Most often the syndrome is accompanied by a number of autoimmune pathologies of connective tissue - dermatomyositis, scleroderma, and in such cases is called - secondary Sjogren's syndrome. If the pathology develops independently, the name sounds like a primary Sjögren's syndrome, or Sjogren's disease.
What it is?
Sjogren's syndrome - an autoimmune systemic failure of the connective tissue, which manifests itself in the involvement pathological process exocrine glands, mainly salivary and lacrimal and chronic progressive over.
morbid anatomy
The main morphological feature is the infiltration of the exocrine glands and lymph plasma cells. The first to suffer the lacrimal and salivary glands, a little less - glands of the bronchi, digestive tract and vagina.
- It affects both large and small gland. First, at an early stage of the disease, the process involved is only small ducts; the progression of its infiltration extends further, to the actual breast tissue, resulting in glandular tissue atrophies and is replaced by connective. In some cases infiltrates described above occur not only in the exocrine (exocrine) glands, but also in other organs and body systems, in particular the muscles, lungs, and kidneys. This ultimately leads to dysfunction of the affected organ.
- In 30-40% of patients in the material, taken by biopsy salivary glands, metaplasia determined (modification) of cells lining the ducts: myoepithelial appearing islets.
Slices of the affected glands are destroyed in some patients, while others lobular structure preserved. Glands or increased in size, or are in the normal range.
What's interesting, even in the absence of a bright clinical symptoms of Sjogren's syndrome in a patient in any connective tissue disease likely to be found out histological signs of inflammation of the salivary glands.
Causes of
The causes of Sjogren's syndrome have not been established until the end. Among the most likely is the theory of abnormal reaction of the immune system. Such a reaction develops in response to damage to the cells of glands of external retrovirus, in particular, it - Epstein-Barr virus, VI of the herpes virus, cytomegalovirus, human immunodeficiency virus. Despite the significant similarity of immunological disorders with changes in the body affected by the virus, direct evidence for the role of the virus as a cause of pathology, is not obtained.
Viruses themselves and changed from their influence gland epithelial cells are perceived by the immune system as antigens (foreign agent). The immune system produces antibodies against such cells and gradually causes tissue destruction gland. The disease often occurs as a hereditary or family, particularly common among twins, which suggests that there is a genetic predisposition.
Thus, it is believed that the mechanism of development of the pathology and is essential combination of a variety of factors:
- stress reactions of the organism, which occurs due to the immune response;
- immune regulation involving sex hormones, as evidenced by a rare disease among people under 20 years, while children often get sick girl;
- immune surveillance via T-lymphocytes;
- viral;
- genetic.
There are two types of Sjogren's syndrome: primary - symptoms of the disease are the first manifestations of it, and the secondary, when the symptoms manifested in patients suffering from other rheumatic diseases, such as scleroderma, rheumatoid arthritis or systemic lupus erythematosus. The primary and secondary embodiments syndrome occur approximately with the same frequency. It is sometimes difficult to accurate diagnosis. Sjogren's syndrome is quite common: in the UK, for example, there are about half a million patients. The most commonly affects women between the ages of 40 to 60 years, whereas only one patient in the 13 - man.
Symptoms of Sjogren's syndrome
Symptoms of the disease is divided into two groups:
- Glandular manifestations that affects epithelial cancer and impairs their function.
- Vnezhelizistye manifestations. The symptomatology of this group is very different, due to the defeat of various human organs.
Glandular symptoms are the following:
- Pathology of the lacrimal glands. In this case, there are unpleasant and painful sensations in the eyes (burning eyes seemed begins to cut, sensation of sand in them), while there is a characteristic itching around the eyes, redness. As a result, vision is reduced, the occurrence of dot bleeding, swelling, sensitivity to light, eye pain.
- Pathology of the salivary glands. Manifestation of inflammation primarily of the parotid glands, which increased in size, sometimes with the appearance of symptoms. Also occurs dryness of the oral mucosa, ingestion of food is difficult, often patients have food drink water, due to the problem of swallowing. Thus oral mucosa takes a bright pink color.
- The dryness of the respiratory tract, which results in inflammation of the bronchi, trachea, lungs.
- Skin disorders, resulting in the skin becomes dry.
- Defeat the nasal mucosa. The appearance of dryness in the nose, intranasal crust coating, whereby the inflammation.
Vnezhelizistym manifestations characterized by the following features:
- The emergence of tracheobronchitis, accompanied by cough, shortness of breath. Often during the examination of the patient revealed pneumonia or pulmonary fibrosis.
- Swollen lymph nodes.
- Disorders of the peripheral nervous system. Thus begin to manifest pain (tingling, burning).
- The elevated temperature;
- Pain in joints and muscles.
- thyroid lesions. It is a rare disorder in which begin to manifest allergic reactions to various foods, medicines, and other means.
- Enlarged lymph nodes (submandibular, cervical, occipital). Often there is an increase in liver and spleen.
- Inflammation of the blood vessels, which takes place on handicap atherosclerosis of the lower limbs due to impaired circulation. When functioning tackle vessels, skin diseases manifest themselves (eruption), which are accompanied by itching, burning and elevated temperature.
Often against the background described by us in patients with pathology develops increased individual sensitivity to certain drugs, Specifically, a non-steroidal anti-inflammatory drugs, some antibiotics (penicillin), the so-called basal drug therapy cytostatics.
Diagnostics
Burning eyes and dry mouth may not always mean that it is the presence of a person of this syndrome. Diagnose Sjogren's syndrome can be only in the presence of inflammatory lesions glands. However, there are occasions when a similar result may cause various metabolic diseases in which salivary secretion is reduced considerably (it usually diabetes).
As a result, people age prematurely reduced salivary and lacrimal glands. These forms of dry eye and mouth have nothing to do with Sjogren's syndrome. Also, for the diagnosis of this syndrome is carried out a study of tissues. For this type of research taking small fragments of the oral mucosa, which examined with a microscope. Thus set mucous glands.
complications
Common effects of Sjogren's syndrome:
- lymphoma (tumors affecting blood, lymph nodes);
- vasculitis (inflammation of the blood vessels which can occur everywhere);
- accession secondary infection;
- development of cancer;
- inhibition of blood formation, reduction of blood leukocytes, erythrocytes and / or platelets.
If the patient for a long time does not take any measures for the treatment of Sjogren's disease or the wrong therapy was appointed, the pathology progresses, leading to serious violations of organs and systems.
Sjogren's Syndrome Treatment
In the presence of Sjogren's syndrome treatment is performed depending on the stage of the disease and the presence of systemic manifestations.
In order to stimulate the glands function is carried out:
- drip kontrikala.
- subcutaneous administration of galanthamine.
- with the purpose of bracing held vitamin courses.
- as symptomatic treatments are assigned "artificial tears" (eye drops), - a low viscosity - Lakrisifi (200-250rub) Tear natural (250 rubles) Lakrisin medium viscosity, high viscosity Oftagel 180 RUB RUB 200 Vidisik, Lakropos 150 rubles.
In the initial stages in the absence of lesions and other systems unexpressed laboratory changes appointed prolonged courses of corticosteroids (prednisone, dexamethasone) in small doses.
If signs and laboratory parameters significantly expressed, but there is no systemic manifestations, to corticosteroids added cytotoxic immunosuppressive drugs - cyclophosphamide, hlorbutin, azathioprine. Supportive therapy is carried out by the same means in a few years.
- In the presence of lesions of systemic symptoms regardless of the stage of the disease once assigned in high doses of corticosteroids and immunosuppressive drugs for a few days with a gradual transfer to the support dose.
- When generalized polyneuritis, vasculitis, kidney disease and other severe manifestations of the disease to the above treatment are added to such methods, in vitro treatment - plasmapheresis, hemosorbtion, plasma Ultrafiltration.
- Other preparations are prescribed depending on complications and concomitant diseases - cholecystitis, gastritis, pneumonitis, endocervicitis and others.
In certain cases, necessary to observe dietary restriction and exercise.
prevention
For the prevention of violations of all the instructions of the attending physician must be observed to prevent exacerbations. Also, a few simple rules must be observed:
- limit the burden on the organs of vision;
- if the syndrome is caused due to another disease, you need to treat the cause of the occurrence.
- avoid stressful situations.
Forecast
Sjogren's syndrome can damage vital organs with the transition to a stable state, a gradual progression, or, on the contrary, long-term remission. This behavior is characteristic of other autoimmune diseases.
- Some people may have mild symptoms of dry eyes and mouth, while others develop serious complications. One patient completely helps symptomatic treatment, others have to constantly deal with the deterioration of view, permanent discomfort in the eye, often recurrent infections of the oral cavity, parotid gland swelling, difficulty in chewing and swallowing. Constant fatigue and joint pain seriously reduce the quality of life. In some patients in the pathological process involved the kidneys - glomerulonephritis, leading to proteinuria, violation kontsentrantsionnoy ability of the kidneys and distal renal tubular acidosis.
- Patients with Sjögren's syndrome have a higher risk of non-Hodgkin's lymphoma compared with healthy people and people living with other autoimmune diseases. In approximately 5% of patients develop some form of lymphoma.
In addition, it was found that children of women with Sjögren's syndrome during pregnancy, the higher the risk of neonatal lupus erythematosus with congenital heart block.
