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Sjögren Syndrome is a chronic disease that progresses very slowly over time. Because of this, there may be a period of up to 10 years between the onset of symptoms and the diagnosis of the disease. As these symptoms usually appear in middle-aged women, they can be seen as normal ageing or menopausal symptoms (e.g. vaginal dryness), further delaying a diagnosis.
The most serious aspect of Sjögren Syndrome is the increased risk of developing haematological cancer (lymphoma), which is estimated to be 10 to 30 times greater than in the general population.
One of the main breakthroughs are the international indices that measure the activity of the disease (known as ESSDAI and ESSPRI), particularly acute complications in the main organs affected. These involve a detailed analysis and definition of all the symptoms a Sjögren Syndrome sufferer may present, beyond just dryness (so-called systemic or extra-glandular manifestations). The aim of this project is to evaluate and diagnose systemic manifestations in the same way at an international level, and to help define a more accurate and precise diagnosis, better treatment, and a better prognosis.
It is highly recommended that the specialist controlling the disease use the ESSDAI index to classify the risk of complications (the higher the score, the greater the risk).
Fertility and offspring are generally not affected by the presence of Sjögren Syndrome, with one important exception: the passage of the mother's antibodies (anti-Ro) through the placenta can lead to heart problems in the foetus, or skin lesions in the newborn.
It is very important that, during pregnancy, the woman is monitored by a multidisciplinary team of specialists (autoimmune disease specialist, obstetrician/gynaecologist, and cardiologist or dermatologist) with experience in evaluating patients with autoimmune diseases.
The main chronic complications of the disease derive from prolonged periods of hydration problems affecting the main mucous membranes. This leads to many symptoms that become chronic, including itchy eyes, a dry mouth, dry cough, or tiredness and chronic pain.
If certain systemic conditions are not detected and treated quickly enough, the patient may develop chronic organ failure, for example, of the kidneys (chronic renal failure) or lungs (pulmonary fibrosis).
Lymphoma is the most serious complication of the disease, and leading specialists understand the factors that can help diagnose this cancer at an early stage.
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Substantiated information by:
Manuel Ramos Casals
Published: 28 May 2019
Updated: 28 May 2019
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