Is Gougerot-Sjögren syndrome fatal?

Gougerot-Sjögren syndrome is an autoimmune disease characterized by a reduction in secretions of tears and saliva leading to a dry syndrome that can affect different organs and viscera. This is’a rare disease affecting just under one in 10,000 adults and “mainly the women” after 40 years, informs us Dr Rakiba Belkhir, rheumatologist at Bicêtre hospital. “We have studied a lot the role of sex hormones to try to understand this difference in prevalence between women and men but for the moment we do not have the answer. What we know is that there is often a genetic terrain, that is to say thatwithin the same family of patients, some will have Gougerot-Sjögren syndrome, others will have different autoimmune diseases. It is a polygenic disease, which means thatthere are lots of genes that are incriminated and when you have it, you don’t systematically pass it on to your children“, specifies the rheumatologist. Gougerot-Sjögren syndrome, also called autoimmune sicca syndromeis a rare sickness which is characterized by the presence of autoantibodies, that is to sayantibodies that are directed against our own cells. The disease can appear, during stress, pregnancy or a viral infection.

What are the symptoms ?

The disease causes a reduction or even complete cessation of the secretion of certain glands of the human body, notably the salivary, lacrimal, vaginal and digestive glands. This is responsible for dry skin, dry eyes, dry mouth. The eye symptoms are tingling of the eyes, feeling of grain of sand in the eyes, absence of tears, recurrent keratitis. Dry mouth and/or eyes are debilitating, requiring the patient to drink at night and often put lubricating drops in the eyes. Other symptoms are pain (particularly joint pain) and fatigue. “A biopsy of the small salivary glands in the lower lip (under local anesthesia) will help with the diagnosis by looking for inflammation at this level.” comments Dr Rakiba Belkhir. This syndrome can be isolated or associated with other autoimmune pathologies such as rheumatoid arthritis or systemic lupus erythematosus.

Lymphoma risks?

Lymphoma occurs in 5% of patients, which means that 5 out of 100 people could develop lymphoma. This is a very specific lymphoma which particularly affects the parotid, the most important salivary gland in the body, and which generally has a very good prognosis. “These are so low grade lymphomas that many doctors don’t treat, they just monitor them. Other teams treat them with light chemotherapies like it is a mildly aggressive lymphoma. Lymphoma is a reflection of the disease which indicates that Sjögren’s syndrome is very active on the immune level, it develops on this occasion“, reassures the rheumatologist.

What are the treatments ?

The treatment of this disease remains difficult, there is no cure. The proposed symptoms target the symptoms. “It’s a disease that will affect patients very differently. Some will only have pain and dry mouth and eyes. Others will develop more severe forms which will affect the lungs or kidneys.explains the specialist. Many trials have been done with drugs that are used in autoimmune diseases, including Platénil®, but it does not work on everyone so we do not have a reference treatment. Hence the importance of the clinical trials which are currently underway. We will just take care of the symptoms: pain with anti-inflammatories and painkillers, dry eyes with eye drops, dry mouth with pilocarpine. “.

What life expectancy?

The syndrome impairs the quality of life of patients but has no impact on the vital prognosis in the absence of more serious complications. The main systemic complication is the occurrence of lymphoma which is approximately 10 times more frequent than in the general population. However, its frequency remains low: 5% of patients

Risks if you are pregnant?

“Sjögren’s syndrome does not pose a particular problem for the patient during pregnancy in the sense that she will not have more relapses. On the other hand, it can have a negative effect on fetal heart function when we discover SSA antibodies (dry A syndrome) before and during pregnancy”, warns Dr Rakiba Belkhir. Special monitoring will therefore be instituted, in particular by fetal echocardiography in the second and third trimester to detect this anomaly. If it is proven, treatments will be put in place. The main risk for the child is having a pacemaker from birth to improve heart conduction.

Secondary Gougerot-Sjögren syndrome

We speak of secondary Sjögren syndrome when it is secondary to another autoimmune disease. For example, rheumatoid arthritis which is the most common autoimmune joint disease in France. “Often, we say rheumatoid arthritis with secondary Sjögren’s syndrome. For us, this simply means that polyarthritis is predominant in the patient. We are currently working to find out if secondary Gougerot-sjögren syndrome is really different from primary but for the moment, we do not know“, continues the specialist.

Thanks to Dr Rakiba Belkhir, rheumatologist at Bicêtre hospital, national reference center for Gougerot-Sjögren syndrome.