Sjogren’s syndrome

Sjögren syndrome: description

Sjögren syndrome is an autoimmune disease that damages the salivary and lacrimal glands of the body for many years. The reason is that the immune system is acting against these structures and destroying them. In addition, patients suffering from Sjögren’s syndrome suffer ten times more frequently from allergies and gluten-sensitive enteropathy (gluten intolerance in cereals) than the average population.

The Sjögren syndrome is named after a Swedish ophthalmologist, who first described the disease in 1933. Because Sjögren syndrome has symptoms of various kinds that appear and are related at the same time, physicians use the word “syndrome”. One can also speak of a symptom complex.

Sjögren syndrome: who is affected?

As with other autoimmune diseases, women are more likely to suffer from Sjögren syndrome than men. It is estimated that 19 out of 20 patients are female. The symptoms start on average around the age of 45, but can occur much earlier or later. Every year about four out of every 100,000 people get Sjögren syndrome. Overall, it is estimated that in Germany 0.2 percent of the population are affected.

Primary and secondary Sjögren syndrome

Doctors distinguish between a primary and a secondary Sjögren syndrome. The primary Sjögren syndrome develops without any other disease causing it. Patients with Sjögren’s syndrome have previously had other conditions that promote the development of Sjögren’s syndrome. These include, for example, inflammatory rheumatic diseases or virus infections.

Sjögren syndrome: symptoms

The Sjögren syndrome is characterized by two main symptoms:

• Dry eyes
• Dry mouth

The dry eyes give the patient a foreign body sensation in the eye. Many sufferers report the impression of constantly observing grains of sand.

Due to the lack of memory production, many patients have to drink during the meal in order to swallow the food better. In addition, the dry mouth causes a constant feeling of thirst.

Therefore, the Sjögren syndrome is also referred to as sicca syndrome, because “siccus” means in Latin “dry”. In addition to the salivary and lacrimal glands, other glands of the body may be affected by the disease. Some women also suffer from vaginal dryness in Sjögren syndrome.

The Sjögren syndrome is not only noticeable in body glands. Many sufferers also suffer from the following symptoms:

• Fatigue, fatigue
• Depression, lack of concentration
• indigestion
• Muscle and joint pain
• Circulatory disorders of the fingers (Raynaud’s phenomenon)

The symptoms of Sjögren syndrome can be very diverse and vary greatly in severity from patient to patient.

Sjögren syndrome: causes and risk factors

Sjögren syndrome is an autoimmune disease. This means that parts of your own immune system (immune system) are directed against the body. In Sjögren’s syndrome, antibodies are formed that attack various tissues. They are called auto-antibodies. In addition, inflammatory cells (lymphocytes) enter the body glands. They cause inflammation here, and the glands can no longer perform their tasks such as the production of tear or saliva. This results in dry eyes and dry mouth.

Why and exactly how Sjögren Syndrome comes about is currently being researched. So far, there are some indications of possible connections. Thus, observations show that the disease occurs familial. This suggests that the tendency to Sjögren syndrome can be inherited. It also discusses whether changes in the hormonal balance, such as menopause, are a trigger for the disease. Stress could also be a cause of Sjögren syndrome.

Secondary Sjögren’s syndrome can occur when patients previously had other illnesses. These diseases include:

• inflammatory rheumatic diseases (such as rheumatoid arthritis, lupus erythematosus)
• Infectious diseases (such as hepatitis B and C)
• other autoimmune diseases (such as primary biliary cirrhosis, thyroid dysfunction)

Sjögren syndrome: examinations and diagnosis

Until the diagnosis Sjogren’s syndrome is made, often go up to ten years. Because many patients are initially in the treatment of different specialists – depending on which symptoms are in the foreground. Since the individual doctors often do not have an overview of all complaints of the patient, it takes a long time until all these symptoms are combined to form a clinical picture – the Sjögren syndrome.

Sjögren’s syndrome: medical history

To substantiate the suspicion of “Sjögren syndrome”, it is important for the doctor to learn more about your medical history (anamnesis). For this he asks you the following questions:

• Do you feel particularly tired or beaten, even for no apparent reason?
• Do you suffer from rheumatism or any other autoimmune disease?
• Do you often feel that there is a foreign object in your eye, such as a grain of sand?
• Do you need to drink a lot during meals to be able to swallow well?
• Does anyone in your family suffer from Sjögren syndrome?

Sjögren syndrome: examinations

To determine whether your lacrimal glands produce enough tears, the ophthalmologist performs the so-called Schirmer test. For this purpose, a litmus paper strip is placed in the corner of your eye for five minutes. It is then read how much the strip was soaked through the tear fluid. In addition, your ophthalmologist can shine a so-called slit lamp into your eye and thus detect a possible inflammation of the cornea (keratitis).

In the ear, nose and throat doctor is mainly the investigation of salivary glands in the foreground. On the one hand, the Saxon test can be carried out. The patient takes a cotton ball for two minutes in the mouth. The piece of cotton is then weighed to determine the amount of saliva produced. On the other hand, the ENT doctor examines the salivary glands by means of ultrasound or scintigraphy (a nuclear medicine procedure). In this case, inflammatory changes in the gland ducts can be detected.

Important in the diagnosis of Sjögren syndrome is also a blood analysis. In the process, antibodies are sought that have formed the immune system and attack the body’s own structures (autoantibodies). However, they are not detectable in all patients with Sjogren’s syndrome, but only in about 70 percent. In addition, other blood levels are determined which may also indicate Sjögren syndrome, such as rheumatoid factors (present in about half of the patients).

In addition, after local anesthesia, a pinhead-sized tissue sample can be removed from the lower lip and examined under a microscope. When Sjögren syndrome is present, the pathologist sees inflamed cells in the glands. This examination is also called histological backup.

Sjögren syndrome or other disease?

Before you are diagnosed with Sjogren’s syndrome, your doctor must rule out many other causes that produce similar symptoms. These include:

• sarcoidosis
• Virus infections (such as viral hepatitis, HIV, Epstein-Barr virus, mumps)
• Graft-versus-host disease after a transplant
• amyloidosis
• tumor diseases
• old age
• radiotherapy
• Side effects of medications such as antihistamininka or tricyclic antidepressants
• limited amount of drink
• Vitamin A deficiency
• dry air

Sjögren syndrome: treatment

Once the diagnosis “Sjögren syndrome” is established, you should seek treatment from a rheumatologist. These specialists usually have the best experience with Sjögren’s syndrome. Your rheumatologist can also serve as an interface and specifically refer you to other specialists if certain symptoms are to be treated. This includes, for example, the treatment at the dentist or ophthalmologist. But also gynecologists, ear, nose and throat doctors, pain therapists, lung specialists or specialists in kidney disease can be involved in the treatment.

Sjögren syndrome: therapy strategy

Sjögren syndrome therapy is symptomatic. This means that the symptoms that occur, such as dry eyes, are relieved. These include:

• in dry eyes: eye drops or pilocarpine, which stimulates the lacrimal glands to produce fluid
• in muscle and joint pain: pain medications such as ibuprofen or acetylsalicylic acid, anti-inflammatory agents such as cortisone or hydroxychloroquine
• In the case of damage to internal organs or blood vessels: drugs that suppress the immune system (immunosuppressants) such as azathioprine or methotrexate

If a secondary Sjögren syndrome is present, the underlying disease is first treated with the intention that the Sjögren syndrome then also disappears.

Sjögren syndrome: what you can do yourself

You can also do a few things yourself to positively influence your health:

• Pay attention to a careful tooth cleaning. The reduced production of saliva favors tooth decay. Check with the dentist regularly for checks.
• Inhale regularly with saline or use nasal douche to reduce the risk of upper respiratory tract infections.
• Chew gum to stimulate the salivary glands to produce saliva.
• Drink more than two liters of water a day.
• Protect your eyes with sunglasses from sun and wind.
• Some patients have to change their diet and lifestyle in Sjögren syndrome. This mainly affects people who additionally have a gluten intolerance.

Sjögren syndrome: disease course and prognosis

The Sjögren syndrome is a chronic disease: it progresses and can not be cured yet.

Some patients are affected by glands and joints as well as other parts of the body. As a result, Sjögren syndrome can further limit life expectancy and quality of life. Affected can be:

• internal organs (pneumonia, nephritis)
• Blood vessels (vasculitis)
• Nervous system (inner ear deafness, peripheral neuropathy, encephalopathy)

Another possible complication of Sjögren’s syndrome is the corneal ulcer of the eye. It can lead to blindness. In addition, Sjögren syndrome promotes the development of a so-called non-Hodgkin’s lymphoma. This cancer is based on cells of the immune system and is found in the Sjogren’s syndrome heaped in the parotid gland.