Sjögren's syndrome is a systemic autoimmune disease, also known as "dry syndrome". The disease was named after a Swedish ophthalmologist who in 1929 treated a patient who suffered from dry mouth, eyes and joint pain. We will talk about what kind of disease it is, what are its causes and symptoms, as well as its treatment.
Syndrome Information
Sjögren's syndrome is more common in women than in men, occurring nine times out of ten. As a rule, such a syndrome affects women immediately after menopause. But in general, the disease can affect people of absolutely any gender, regardless of age. There are no global statistics for this pathology, but in developed countries, including Russia, the incidence is estimated in millions. Thus, the disease is one of the most common rheumatic pathologies.
With Sjögren's syndrome, a person's immunity begins to perceivebody cells as foreign dangerous elements, starting to slowly, and at the same time systematically destroy them. Immune cells enter the tissues of the external secretion glands, affecting them, so they, in turn, secrete less of the required secret (saliva, tears, etc.).
In addition, the disease often affects other organs, provokes arthralgia along with pain in the joints and muscles, causing shortness of breath in a person. There is also a syndrome that accompanies rheumatoid arthritis, diffuse connective tissue diseases, diseases of the biliary system and other autoimmune pathologies.
It is extremely important to consult a doctor already at the first symptoms, otherwise running Sjögren's syndrome can acquire an unfavorable course, affecting vital organs, which often leads to all sorts of complications, and in rare cases even death.
Reason for development
What are the main causes of Sjögren's syndrome?
One of the main factors that triggers this disease is an autoimmune failure. With such a violation, the immune system begins to destroy the cells of the secretion glands. Why exactly this happens is difficult to say, this mechanism needs to be clarified. The causes and symptoms of Sjögren's syndrome are closely related.
Another factor in the occurrence of the disease is a genetic predisposition. Sometimes in the event that such a disease is present in the mother, then it can also be detected in the daughter. Any changes in the female hormonal background can also provoke such a syndrome. As a rule, Sjögren's syndrome (pictured) develops onagainst the background of other systemic pathologies, for example, as part of rheumatoid arthritis, with systemic lupus erythematosus, and so on.
Forms of illness
There are two options for the development of this disease. The clinical manifestations of these varieties are absolutely identical, but there are some differences in the causes of occurrence:
- In the first case, this syndrome may occur against the background of any other autoimmune diseases. Mostly its formation occurs in rheumatoid arthritis.
- In the second case, Sjögren's syndrome is formed as an independent disease.
Directly by the nature of the onset and subsequent course, the syndrome can have the following forms:
- Chronic form. In this case, the course of the disease is characterized mainly by the defeat of the glands. The syndrome begins, as a rule, slowly, without any pronounced clinical manifestations. But gradually it develops, a person develops dry mouth, glands enlarge, their function is disturbed. Involvement of other organs in the pathological process is extremely rare in practice.
- Subacute form of the disease. Against the background of this type of syndrome, its course begins with a pronounced process of inflammation. In this case, the patient may experience a high body temperature along with inflammation of the salivary glands. Joints can also become inflamed. Against the background of all these processes, patients will experience inflammatory changes in blood tests. This form of the disease is usually characterized by a systemic lesion, that is, against its background in the pathological processmany organs and systems of the body are involved.
Consider the symptoms of Sjögren's syndrome.
Symptomatics
All symptoms of this disease can be conditionally divided into extra-glandular and glandular manifestations. Glandular signs of pathology are manifested in a decrease in the production of secrets.
One of the main symptoms of Sjögren's syndrome is inflammation of the eyes, which is associated with a decrease in the amount of eye fluid. At the same time, patients begin to be disturbed by a feeling of discomfort in the form of burning, scratching and sand in the eyes. Along with this, patients often feel swelling of the eyelids along with redness, accumulation in the corners of the eyes of a viscous fluid that has a white tint. At the next stage of the disease, patients may begin to complain of photophobia, and as a result, their visual acuity deteriorates.
The second constant symptom of Sjögren's syndrome is inflammation of the salivary gland, which flows into a chronic form. At the same time, patients complain of dry mouth, in addition, an increase in the salivary glands. At the very beginning of the disease, there may be only a slight or intermittent feeling of dry mouth, which appears solely as a result of excitement or physical exertion. Then the dryness becomes permanent. Against the background of such processes, the mucous membrane and tongue dry significantly, acquiring a bright pink color and very often becoming inflamed. In addition, with this symptomatology, dental caries quickly begins to progress in patients. Sometimes, before these signs appear, patients experience an unreasonable increaselymph nodes.
The late stage of the disease is characterized by severe dry mouth, it can be very difficult for a person to talk and swallow solid food. To make this possible, you have to drink water with food. Cracks appear on the lips. Chronic gastritis with insufficient secretion may also occur, which will be accompanied by belching and a decrease in appetite, and the appearance of nausea is not excluded. In every third patient at a late stage, as a rule, doctors note an increase in the size of the parotid glands.
Among other things, there may be damage to the biliary tract along with hepatitis and pancreatitis. In the late stage of the syndrome, the nasopharynx becomes very dry, and dry crusts form in the nose. At this stage, the patient may develop hearing loss and otitis media. Due to the presence of dryness in the larynx, hoarseness of the voice occurs. Secondary infections also manifest themselves at this stage in the form of often recurrent sinusitis, tracheobronchitis and pneumonia. Every third patient has an inflammatory process in the genital area.
Many people wonder if Sjögren's syndrome can be treated? More on that later.
Extra-glandular Syndrome Symptoms
Extra-glandular symptoms of the syndrome are quite diverse and have a systemic character. First of all, patients have pain in the joints along with stiffness in the morning, muscle discomfort and muscle weakness. Most patients often note an increase in lymph nodes in the submandibular, occipital, cervical and supraclavicular regions.
In half of patients, doctors observe inflammation of the respiratory system inthe form of dryness in the throat, perspiration, scratching, dry cough and shortness of breath. Sjögren's disease can manifest as cutaneous vasculitis, and skin rashes are also likely, which initially appear on the lower legs, and then move to the abdomen, thighs and buttocks. In addition, the rash is accompanied by skin irritation, burning sensation and high fever.
One in three patients have an allergic reaction to some antibiotic drugs, in addition to B vitamins, foods and washing powder. With Sjögren's syndrome, the appearance of lymphomas is possible. The general situation is aggravated by the fact that this pathology often occurs against the background of other diseases. Most often, it is formed in the presence of certain rheumatic diseases.
Diagnostics
When diagnosing this pathology, it is necessary to take into account not each of its signs separately, but the entire set of symptoms at once. In the event that there are at least four factors, then it is possible to say with a high degree of probability that a person really has Sjogren's syndrome. So, doctors pay attention to the following symptoms:
- Presence of enlarged parotid salivary glands.
- The development of Raynaud's syndrome, in which there is a violation of blood circulation in the legs or hands.
- Appearance of dry mouth.
- Increased erythrocyte sedimentation rate.
- Occurrence of joint problems.
- The occurrence of frequent outbreaks of chronic conjunctivitis.
In order to confirm the presence of the disease, various methods of laboratory diagnosis are used. First of all, blood is donated for the analysis of Sjögren's syndrome. Often, the patient is diagnosed with anemia along with mild leukopenia and elevated ESR. As for the biochemical analysis, it will show an increased level of globulins and total protein. Fibrin will also be contained in an increased amount. Immunological responses will reflect elevated levels of immunoglobulins and abnormal autoantibodies.
What other diagnosis of Sjögren's syndrome is carried out?
As part of the Schirmer test, low production of tear production is usually detected in response to stimulation with ammonia. Thanks to the staining of the sclera with special dyes, it is possible to detect erosion of the epithelium. Diagnostic procedures also include contrast radiography and a number of the following tests, for example:
- Performing a biopsy of the salivary glands.
- Performing salivary gland ultrasound.
- Changing a lung x-ray.
- Performing a gastroscopy procedure.
Among other things, echocardiography is also performed, which helps to identify complications that affect other organs with body systems.
Treatment of Sjögren's syndrome should be comprehensive.
Syndrome Treatment
The main specialist who diagnoses and treats this disease is a rheumatologist. But in the course of treatment, oftenthe help of other doctors is required, for example, a dentist, an ophthalmologist, a gynecologist, a nephrologist, a pulmonologist, and so on. In the treatment of this syndrome, the main place is occupied by treatment with hormonal and cytostatic drugs that have an immunosuppressive effect.
In the presence of ulcerative necrotic vasculitis, glomerulonephritis, polyneuritis and other systemic lesions in the treatment of Sjögren's syndrome, plasmapheresis is considered the most effective. In addition, patients are advised to prevent secondary infections. In order to eliminate dryness in the eyes, artificial tears are used, and solutions with antiseptics are also used for washing. In addition, local medicinal applications are prescribed to eliminate inflammation of the parotid glands.
To relieve dry mouth, artificial saliva is used. Applications using sea buckthorn, rosehip oil also help perfectly. Such oils contribute to the rapid regeneration of the oral mucosa. Directly for the prevention of caries, patients need to be observed by a dentist.
Also, patients with this syndrome may need to consult a specialist gastroenterologist. Patients suffering from secretory insufficiency of the stomach are prescribed long-term replacement therapy with hydrochloric acid. And patients who do not have a he althy pancreas are prescribed enzyme therapy. If left untreated, this syndrome can significantly reduce a person's quality of life. Left unattended, the pathological process can cause certaincomplications that often lead to disability.
We looked at the symptoms and treatment of Sjögren's syndrome.
Complications and consequences
So, as already noted, in the absence of timely and adequate therapy, this disease can steadily progress and, as a result, lead the patient to significant disorders associated with organ damage. The main complications, and at the same time the causes of death, may be:
- Development of vasculitis (inflammation of blood vessels), which can involve many organs.
- The appearance of lymphoma - a malignant disease that affects the lymph nodes with blood.
- Occurrence of other malignant neoplasms. In this case, the stomach most often suffers.
- Development of autoimmune oppression of hematopoiesis with a decrease in the required content of certain cellular elements in the blood, for example, leukocytes, erythrocytes, platelets, and so on.
- Accession of secondary infections.
Prophylaxis
Prevention of this syndrome comes down, first of all, to the prevention of exacerbations and further progression of the pathology. Therefore, as part of the prevention, the following recommendations are required:
- It is extremely important to take your doctor's prescribed medicines regularly.
- Patients should limit the load on the organs of vision, in addition, on the vocal cords.
- Any infections should be prevented.
- Very importantavoid all kinds of stressful situations.
- It is required to exclude the implementation of any vaccinations and radiation therapy, including.
- Physiotherapy should be treated with caution. The use of such is possible only after consultation with a physiotherapist.
- If the syndrome develops against the background of another pathology, the patient, first of all, needs therapy for the underlying disease.
Reviews on Sjögren's syndrome
Reviews about this disease abound. Pathology is rather unpleasant, and if left untreated, it is dangerous. People write that the result will be noticeable only if you follow all the doctor's recommendations and avoid stress.
Sjögren's disease is a chronic inflammatory disease characterized by damage to the secretion glands. In this case, first of all, it is the salivary and lacrimal glands that suffer in humans. This pathology belongs to the group of autoimmune diseases, characterized by certain failures in the immune system. Against the background of these failures, for reasons unknown to date, the body perceives native cells as foreign and produces antibodies to them. Such a failure, in the absence of proper treatment, leads to the development of a chronic inflammatory process that can spread to various organs and systems of the human body, which ultimately can lead the patient to disability. In this regard, when this syndrome appears, it is very important to see a doctor.
The article presented the causes, symptoms, treatment andprevention of Sjögren's syndrome.
