Le Gougerot-Sjögren syndrome (pronounce sjeu-greunne), which is part of the dry syndromes, is a chronic condition of autoimmune origin, i.e. linked to a reaction of the immune system against certain constituents of the body, in this case the exocrine glands, secreting fluids in the skin or mucous membranes.

Its discovery dates back to 1933, by D^r Henrik Sjögren, a Swedish ophthalmologist.

Its manifestations are linked to the infiltration of certain glands by lymphocytes causing the decrease in their secretions. The salivary glands of the mouth and the lacrimal glands are the most commonly affected, responsible for a “dry syndrome”. We can also observe a decrease in sweat, sebum but also infiltration and inflammation in other organs such as the lungs, kidneys, joints or small vessels.

Gougerot-Sjögren syndrome is a rare disease affecting just under one in 10 adults. Women are 000 times more affected than men. It most often occurs around age 10 but can occur earlier around age 50 and 20. 

Types

The disease can manifest itself in 2 ways:

• Primary. The syndrome appears in isolation. This is the case 1 time in 2. About 93% of those affected are women, and symptoms usually appear around the age of 50;
• Secondary. The syndrome is associated with another autoimmune disorder, the most common of which is rheumatoid arthritis.

Causes

The cause of Gougerot-Sjögren syndrome is unknown. However, the disease results from an autoimmune reaction. The reason why the immune system of the body comes to malfunction and attack its own tissues is still nebulous. Several hypotheses are under study. According to the researchers, it is likely that the onset of this syndrome requires both genetic predisposition and the arrival of trigger factors (viral infection, hormonal changes, stress, etc.).

The symptoms

In 2/3 of cases the manifestations linked to the involvement of the exocrine glands are associated with the involvement of other organs (this is called a systemic disease)

Dry eyes and mouth are usually the first to occur. However, they appear later for people who already have arthritis. 

In the eyes, dryness can cause a burning or itching sensation. The eyelids often stick together in the morning, and the eyes are more sensitive to light.

Dry mouth makes speaking, chewing and swallowing more difficult. 

We can also observe a persistent dry cough, joint pain, muscle pain, fatigue

The sicca syndrome can be complicated at the ocular level by blepharitis or keratitis and at the oral level by damage to the gums, cavities, dental mobility, canker sores, oral secondary infections in particular by mycoses. One can observe an hypertrophy of the parotid glands, transient or not.

The extra glandular manifestations concern the joints (one in 2), Raynaud’s syndrome (fingers becoming white in reaction to the cold). Other attacks are more serious but rarer, at the pulmonary, renal, cutaneous or peripheral nerves level. 

Fatigue is very common, and is accompanied by diffuse pain.

Diagnostic

Diagnosis is difficult because the person does not have all the symptoms and these may be related to other conditions or to taking treatment.

Various examinations are essential: search for autoantibodies in the blood (anti-SS-A, anti-SS-B antibodies), an evaluation of the production of the lacrimal glands using a filter paper (Schirmer’s test ), an observation of the thin membrane which covers the eye by staining with rose bengal and a saliva test to evaluate the dryness of the mouth and the demonstration of lymphocytic nodules on a saliva biopsy; performed in the oral salivary glands, this gesture is not very aggressive and painless. The diagnosis is based on the combination of a number of these clinical and biological signs. 

The doctor may also suggest screening for other locations of the disease or other autoimmune pathologies.

At the time of diagnosis, the doctor asks the patient about his general state of health, the types of medications he is taking, and also about the diet and the amount of water and other fluids consumed daily.