Sjögren’s syndrome is a systemic autoimmune disease, also known as “dry syndrome”. The disease was named after the Swedish ophthalmologist, who in 1929 treated a patient suffering from dry mouth, eyes and joint pain. What kind of disease is it, what are its causes and symptoms, as well as its treatment will be described below.
Syndrome Information
In women, Sjogren's syndrome is more common than men, which occurs in nine out of ten cases. Typically, this 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 millionths. Thus, the disease is one of the most common rheumatic pathologies.
With Sjogren's syndrome, human immunity begins to perceive the cells of the body as foreign dangerous elements, starting them slowly, and at the same time, systematically destroying them. Immunity cells enter the tissues of the endocrine glands, affecting them, so they, in turn, secrete less than the required secret (saliva, tears, etc.).
In addition, the disease often affects other organs, provoking 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 diseases of the connective tissues, diseases of the biliary system and other autoimmune pathologies.
It is extremely important to consult a doctor already at the first symptoms, otherwise advanced Sjogren's syndrome can acquire an unfavorable course, affecting vital organs, which often leads to all kinds of complications, and in rare cases, even death.
Development reasons
What are the main causes of Sjogren's syndrome?
One of the main factors that triggers this disease is autoimmune failure. With this violation, immunity begins to destroy the cells of the secretion glands. It is difficult to say why this is happening; this mechanism needs to be clarified. The causes and symptoms of Sjogren's syndrome are closely related.
Another factor in the occurrence of the disease is a genetic predisposition. Sometimes, if such a disease is present in the mother, then her daughter can also identify it. Any changes in the female hormonal background can also provoke such a syndrome. As a rule, Sjogren's syndrome (pictured) develops against the background of other systemic pathologies, for example, in the framework of rheumatoid arthritis, with systemic lupus erythematosus, and so on.
Forms of ailment
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 can occur against the background of any other autoimmune diseases. Mostly its formation occurs with rheumatoid arthritis.
- In the second case, Sjogren's syndrome is formed as an independent disease.
Directly by the nature of the onset and subsequent course, the syndrome can take the following forms:
- Chronic form. In this case, the course of the disease is characterized mainly by damage to the glands. The syndrome begins, as a rule, slowly, without any pronounced clinical manifestations. But gradually it develops, a person has a dry mouth, glands enlarge, their function is impaired. Involvement in the pathological process of other organs in practice is extremely rare.
- 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 high body temperature along with inflammation of the salivary glands. Joints can become inflamed in parallel. 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, many organs and systems of the body are involved in the pathological process.
Consider the symptoms of Sjogren's syndrome.
Symptomatology
All the symptoms of this disease can be divided into extra-iron and glandular manifestations. Glandular signs of pathology are manifested in a decrease in the production of secrets.
One of the main symptoms of Sjogren's syndrome is eye inflammation, which is associated with a decrease in the amount of eye fluid. Patients at the same time begin to worry about a sense 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 of viscous fluid in the corners of the eyes, which 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 worsens.
The second constant sign of Sjogren's syndrome is inflammation of the salivary gland, which flows into a chronic form. Patients complain of dry mouth, in addition, an increase in the salivary glands. At the very beginning of the disease, only a slight or inconsistent sensation of dryness in the mouth can be observed, which appears solely as a result of excitement or physical exertion. Then dryness becomes constant. Against the background of such processes, the mucous membrane and tongue dry significantly, acquiring a bright pink color and very often become inflamed. In addition, with this symptomatology, dental caries quickly begins to progress in patients. Sometimes, before the onset of these symptoms, patients have a causeless increase in lymph 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 food with water. Cracks appear on the lips. Chronic gastritis with insufficient secretion may also occur, which will be accompanied by belching and a decrease in appetite, the appearance of nausea is not excluded. In every third patient at a late stage, as a rule, doctors note an increase in the parotid glands in size.
In addition, biliary tract damage can be observed 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, a hoarseness of the voice occurs. Secondary infections in the form of often recurring sinusitis, tracheobronchitis and pneumonia also manifest themselves at this stage. Every third patient has an inflammatory process in the genital area.
Many people wonder if Sjogren’s syndrome is treated. About it further.
Non-iron symptoms of the syndrome
Non-ferrous symptomatology of the syndrome is quite diverse and has a systemic character. First of all, patients have joint pain along with stiffness in the morning, muscle discomfort, and muscle weakness. Most patients often notice an increase in lymph nodes in the submandibular, occipital, cervical and supraclavicular areas.
In half of the patients, doctors observe inflammation of the respiratory system in the form of dry throat, tickling, scratching, dry cough and shortness of breath. Sjogren's disease can manifest as cutaneous vasculitis, and skin rashes that initially appear on the lower legs, and then go to the stomach, hips and buttocks, are also likely. In addition, rashes are accompanied by skin irritation, burning sensation and high fever.
Every third patient has an allergic reaction to some antibiotic preparations, in addition to B vitamins, food products and washing powder. With Sjogren's syndrome, lymphomas may appear. The general situation is further 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 symptoms individually, but the entire set of symptoms at once. In the event that there are at least four factors, it can be said with a high degree of probability that a person does have Sjogren's syndrome. So, doctors pay attention to the following symptoms:
- The 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.
- The appearance of dryness in the oral cavity.
- Increased erythrocyte sedimentation rate.
- The occurrence of problems with the joints.
- The appearance of frequent outbreaks of chronic conjunctivitis.
In order to confirm the presence of an ailment, various laboratory diagnostic methods are used. First of all, blood is given for analysis of Sjogren's syndrome. Often anemia is detected in a patient along with moderate leukopenia and an increase in ESR. As for biochemical analysis, it will show elevated levels of globulins and total protein. Fibrin will also be contained in an increased amount. Immunological reactions will reflect exceeded levels of immunoglobulins and pathological autoantibodies.
What other diagnosis of Sjogren's syndrome is carried out?
As part of the Schirmer test, a low production of tear production is usually detected in response to stimulation with ammonia. Thanks to the coloring 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 studies, for example:
- Biopsy of the salivary glands.
- Performing an ultrasound examination of the salivary glands.
- Radiography of the lungs.
- Performing a gastroscopy procedure.
In addition, echocardiography is also performed, which helps to identify complications that affect other organs with body systems.
Treatment of Sjogren's syndrome should be comprehensive.
Syndrome treatment
The main specialist who diagnoses with the treatment of this disease is a rheumatologist. But in the process of treatment, the help of other doctors is often required, for example, a dentist, ophthalmologist, gynecologist, nephrologist, pulmonologist and so on. In the treatment of this syndrome, the main place is the treatment with hormonal and cytostatic drugs with immunosuppressive effects.
In the presence of ulcerative necrotic vasculitis, glomerulonephritis, polyneuritis and other systemic lesions during treatment with Sjogren's syndrome, plasmapheresis is considered the most effective. In addition, the prevention of secondary infections is recommended for patients. In order to eliminate dryness in the eyes, an artificial tear is 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 reduce dry mouth, artificial saliva is used. Applications with sea buckthorn and rosehip oil also help. Such oils contribute to the rapid regeneration of the mucous membranes of the oral cavity. For caries prophylaxis directly, patients need to be seen 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 prolonged replacement therapy with hydrochloric acid. And for patients whose pancreas is not healthy, enzyme therapy is prescribed. In the absence of treatment, this syndrome can significantly reduce a person’s quality of life. An ignored pathological process can cause certain complications, which often lead to disability.
We examined the symptoms and treatment of Sjogren's syndrome.
Complications and consequences
So, as already noted, in the absence of timely and adequate therapy, this disease can progressively progress and as a result will lead the patient to significant disorders associated with organ damage. The main complications, and at the same time, the causes of death can be:
- The development of vasculitis (vascular inflammation), which can invade many organs.
- The appearance of lymphoma - a malignant disease that affects the lymph nodes with blood.
- The occurrence of other malignant neoplasms. In this case, the stomach most often suffers.
- The development of autoimmune inhibition of hematopoiesis with a decrease in the necessary content in the blood of certain cellular elements, for example, white blood cells, red blood cells, platelets and so on.
- Joining secondary infections.
Prophylaxis
Prevention of this syndrome is reduced, first of all, to the prevention of exacerbations and further progression of the pathology. Therefore, in the framework of prevention, the following recommendations are required:
- It is extremely important that you take medications prescribed by your doctor.
- Patients should limit the load on the organs of vision, in addition, on the vocal cords.
- Any infections should be prevented.
- It is very important to avoid all kinds of stressful situations.
- It is required to exclude the implementation of any vaccinations and radiation therapy as well.
- Caution should be exercised in physiotherapeutic procedures. The use of such is possible only after consultation with a physiotherapist.
- In the case of the development of the syndrome against a background of another pathology, the patient, first of all, requires treatment of the underlying disease.
Reviews of Sjogren's syndrome
Reviews about this disease are abundant. Pathology is quite unpleasant, and if untreated, it is dangerous. People write that the result will be noticeable only if you follow all the doctor's recommendations and avoid stress.
Sjogren's disease is a chronic inflammatory disease characterized by damage to the secretion glands. In this case, first of all, salivary and lacrimal glands are affected in a person. This pathology belongs to the group of autoimmune diseases characterized by certain malfunctions 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 malfunction 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 to disability. In this regard, with the appearance of this syndrome, it is very important to see a doctor.
The article presented the causes, symptoms, treatment and prevention of Sjogren's syndrome.