Sjögren’s syndrome is an autoimmune disease in which the body’s defenses target its own tissues and cause inflammation. It belongs to the inflammatory rheumatic diseases. Primarily, the tear and salivary glands are affected, but the infections can also spread to muscles and joints.
What is Sjögren’s syndrome?
Sjögren’s syndrome is a disease of the autoimmune system characterized by chronic inflammation. In an autoimmune disease, the body’s defense system is no longer directed against harmful invaders from the outside, such as bacteria or viruses, but against the body’s own tissues. Sjögren’s syndrome belongs to the inflammatory rheumatic diseases and to the group of collagenoses. Two forms of Sjögren’s syndrome are distinguished. If only lacrimal and salivary glands are affected by infections, it is called primary Sjögren’s syndrome. If the symptoms occur as a concomitant of other connective tissue inflammations, such as rheumatoid arthritis or lupus erythematosus, this is called secondary Sjögren’s syndrome. Women are more often affected by Sjögren’s syndrome than men. The disease is among the most common inflammatory diseases.
Causes
The causes of Sjögren’s syndrome are not clearly understood. On the one hand, it is suspected that the predisposition to the disease is inherited; on the other hand, environmental factors or hormone imbalances are considered possible triggers. Other conceivable causes include medication, viral infections or special stresses on the body, such as pregnancy. It is assumed that the body is in some way misprogrammed by these processes and forms antibodies that attack the body’s own tissue. Another explanation is that in Sjögren’s syndrome, the immune system loses the ability to distinguish between cells that are foreign to the body and those that are the body’s own. This ability is called immune tolerance. However, the reason for the loss of this immune tolerance is not yet known.
Symptoms, complaints, and signs
Patients with Sjögren’s syndrome suffer primarily from dry mucous membranes. Dry eyes are therefore one of the leading symptoms of the disease. Because of the dryness, those affected have the feeling of having a foreign body in their eye. The eyes itch, are reddened and painful. However, not only the eyes, but also the mouth shows a pronounced dryness. Saliva production is severely restricted, so patients have to drink more while eating. This is the only way they can swallow the chewed food at all. Due to the dry mouth, they also experience a constant feeling of thirst. In addition to the salivary and lacrimal glands, other bodily glands can also be affected. For example, some patients suffer from vaginal dryness. The feeling of dryness, burning and itching as well as discomfort during sexual intercourse are the result. Sjögren’s syndrome may also be manifested by non-specific general symptoms. Those affected are constantly fatigued and tired. This is also referred to as fatigue. They have difficulty concentrating and have pain in the joints and muscles. Digestive problems may also occur. Another symptom of Sjögren’s syndrome is Raynaud’s phenomenon, also called Raynaud’s disease. It involves circulatory problems in the fingers that are accompanied by numbness and/or pain.
Diagnosis and course
Two types of symptoms are distinguished in Sjögren’s syndrome. If the immune system targets only the salivary and lacrimal glands, the symptoms are called glandular (affecting the glands). This is the case in primary Sjögren’s syndrome. When the defense also attacks other types of tissue, i.e., when secondary Sjögren’s syndrome is present, the symptoms are called extraglandular (lying outside the glands). The glandular symptoms are mainly manifested in a dry mouth and dry eyes, which physicians call sicca syndrome (sicca = dry). Other mucous membranes such as the throat, nose or vagina may also be affected by the dryness. The extraglandular symptoms are circulatory disorders with the formation of red skin spots, inflammation of joints and fatigue. The symptoms usually develop after the age of 40, but since the malfunctioning of the immune system develops slowly, it is believed that the disease begins after the age of 20 or 30. The symptoms, however, only become noticeable and visible later.The first suspicion of Sjögren’s symptom already arises from the typical symptoms. In order to be able to make a reliable diagnosis, blood tests are carried out. If Sjögren’s syndrome is present, certain antibodies, rheumatoid and inflammatory factors can be detected in the blood.
Complications
Sjögren’s syndrome leads to many different symptoms. First and foremost, affected individuals suffer from very dry eyes. Since the complaints are not particularly characteristic and do not directly indicate the disease, there is usually no early diagnosis and treatment. Those affected continue to suffer from a dry mouth and very pronounced fatigue. Fatigue and a general feeling of illness can also occur due to the disease and have a very negative effect on the patient’s quality of life. However, the disease also affects the muscles and joints, leading to inflammation and severe pain. If the pain also occurs at night, this can lead to sleep problems and irritability or depression in the patient. Restrictions in movement and thus in everyday life can also occur due to Sjögren’s syndrome. Unfortunately, a causal treatment of the syndrome is not possible. However, the symptoms can be well limited with the help of medications and therapies. Particular complications do not occur. Whether there is a reduced life expectancy due to the disease, unfortunately, cannot be universally predicted.
When should you see a doctor?
Since Sjögren’s syndrome is a genetic disease, it must always be treated by a physician. Should the affected person desire to have children, genetic testing and counseling can also be performed to prevent recurrence in descendants. Since Sjögren’s syndrome can lead to serious symptoms and complications, a doctor should always be consulted if symptoms are indicative of this syndrome. A doctor should be consulted for this syndrome when dry and reddened eyes occur. In many cases, sufferers have a permanent feeling that they have a foreign body in their eye. Persistent itching in the eyes may also indicate this condition. In women, Sjögren’s syndrome may be manifested by vaginal dryness, and a doctor should be consulted for this complaint as well. Digestive problems or circulatory problems are also indicators of Sjögren’s syndrome. If the syndrome is suspected, a general practitioner can be consulted. Further treatment is then usually carried out by a specialist.
Treatment and therapy
Treatment of Sjögren’s syndrome is aimed at alleviating symptoms, as there is not yet a curative therapy for autoimmune diseases. Attempts are made only to suppress the immune system to prevent further attacks on the patient’s own body. This is done with the help of drugs such as cortisone. For dryness in the eyes, ointments or drops are prescribed. For the dry mucous membrane of the mouth, there are special mouth rinses or gels. Saliva production can also be stimulated by sucking candies and taking small sips of water throughout the day to moisten the mucous membranes. If the symptoms are particularly pronounced, medications are used to stimulate tear and saliva production. In addition, careful oral hygiene is necessary, as there is an increased risk of caries in Sjögren’s syndrome. If secondary Sjögren’s syndrome is present, the underlying disease and the inflammatory processes in the joints are also treated. Here, pain-relieving and anti-inflammatory drugs are used. Overall, the treatment of Sjögren’s syndrome usually requires the involvement of several specialists such as a dentist, rheumatologist, ophthalmologist, gynecologist and ear, nose and throat specialist.
Prevention
Prevention of Sjögren’s syndrome is not possible, especially since no exact causes are yet known. However, one can strengthen the immune system through a healthy lifestyle with sufficient exercise and a balanced diet.
Follow-up
Since it is a genetic disease, it usually cannot be completely cured. Therefore, affected individuals should consult a doctor at the first symptoms of the disease and initiate treatment to prevent the occurrence of further symptoms and complications.An independent cure cannot be achieved. If a child is desired, genetic testing and counseling should be performed in any case to prevent a recurrence of the syndrome in the descendants. Most sufferers of this disease are dependent on various surgical interventions through which the symptoms can be alleviated and limited. In any case, the affected person should rest and take care of his body after such an operation. Physical exertion or stressful activities should be avoided in order not to put unnecessary strain on the body. Furthermore, the help and support of one’s own family is usually very useful. This often prevents and limits the development of depression and other psychological upsets. In some cases, Sjögren’s syndrome can also reduce the life expectancy of the affected person. However, the further course of this disease is highly dependent on the time of diagnosis, so a general course cannot be given.
What you can do yourself
The disease course of Sjögren’s syndrome is characterized by chronic inflammation. In everyday life, to support the immune system, the design of lifestyle should be optimized. The body’s own defense system can be mobilized by a balanced and vitamin-rich diet. Refraining from the consumption of harmful substances such as alcohol and nicotine is helpful in coping with the disease. In addition, overweight should be avoided and sufficient exercise has a beneficial effect on health. The organism should be protected from further infections. Especially in times of changing weather or seasons, protective measures should therefore be taken in good time and risks of infection should be minimized. Since sexual contact with a partner can cause inconvenience, the partner should be informed in good time about the disease and the existing symptoms. This avoids unpleasant situations in everyday life and prevents misunderstandings. If the affected person needs emotional support in coping with the disease, psychotherapeutic treatment can help. In addition, mental strength should be strengthened, as emotional problems inevitably spread to the entire organism. Frequently, disturbances of blood circulation occur. For this reason, the adoption of a rigid posture should be refrained from, and compensatory movements should be performed at the first sensory disturbances on the skin.