What is Sjögren Syndrome?

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Sjögren Syndrome is a systemic autoimmune disease. Autoimmune because the immune system, responsible for defending the organism from external aggression, reacts against the body itself, producing so-called "autoantibodies" and injuries to its own structures. The reasons for this are not known. In the case of Sjögren syndrome, the organism attacks the exocrine glands that are responsible for producing liquids such as saliva, tears, secretions from the larynx, trachea, and vagina, liquids that hydrate, lubricate and soften the parts of the body that are in contact with the outside, the so-called mucous membranes that are the tissues covering the mouth, nose, eyes, and so on.

The damage that the body’s immune system produces in the exocrine glands incapacitates and prevents them from manufacturing these liquids correctly. This means the patient suffers symptoms of dryness in these areas.

It is a systemic disease because, in addition to the dryness, it can affect any organ in the body and produce a wide variety of symptoms and effects.

Classification of Sjögren Syndrome

Sjögren Syndrome is classified into two types depending on whether or not the disease is accompanied by other autoimmune diseases:

Boca seca, xerostomía

Primary Sjögren Syndrome. This appears without the presence of another associated autoimmune disease.

Man with skin blemishes

Sjögren Syndrome associated with other diseases. The term secondary Sjögren Syndrome, which has been used for years as a result of a translation from English, is a poor term because the word "secondary" seems to indicate that it is something unimportant, or caused by another disease.In this case, the patient, in addition to Sjögren, suffers other autoimmune or rheumatological diseases. The most frequent are systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, and inflammatory myopathies. Sjögren Syndrome may also be associated with autoimmune diseases that affect a single organ ("organ-specific"). The most common are thyroiditis, primary biliary cholangitis, and autoimmune hepatitis.

How many people does Sjögren Syndrome affect?

It can appear at any age and in either sex, but it is more frequent in women over 40, in a ratio of up to 15 women for each man affected by the disease.

The prevalence of the disease, which is its frequency in the population, is around 0.1-0.01% (i.e., one in every 1,000-10,000 people could have it), making it the most frequent systemic autoimmune disease.

Approximately 20-30% of patients with rheumatoid arthritis, systemic lupus erythematosus, or systemic sclerosis may have associated Sjögren Syndrome.

Substantiated information by:

Manuel Ramos Casals

Published: 28 May 2019
Updated: 28 May 2019

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