- What is it?
- Causes
- Symptoms
- Diagnosis
- Treatment
- Evolution of the disease
- Living with the disease
- Research
- Frequently asked questions
Frequently Asked Questions about Sjögren Syndrome
Nine out of ten patients with Sjögren Syndrome are women. The average age to be diagnosed is from 40 upwards, although it can occur in all age groups, including children, and in both sexes.
The most common symptoms are dry eyes, a dry mouth, fatigue and musculoskeletal pain. However, no two people have exactly the same set of symptoms, so patients should remember to share all of their symptoms with their specialist in order to get a proper diagnosis.
This disease is often undiagnosed or misdiagnosed. The symptoms of Sjögren Syndrome can mimic those of the menopause, side effects of medications, allergies, or other medical conditions such as lupus, rheumatoid arthritis, fibromyalgia, chronic fatigue syndrome, and multiple sclerosis. Because all the symptoms are not always present at the same time and because Sjögren Syndrome can affect several organs, doctors sometimes treat each symptom individually and do not recognise the presence of a systemic disease. The average time from symptom onset to diagnosis is 3 to 5 years.
Sjögren Syndrome can be difficult to diagnose. There is no single test that confirms the diagnosis and the disease can appear in many different forms in different patients. It is essential to get care from a specialist in systemic autoimmune diseases who has clinical experience of this disease.
Currently, there is no cure for Sjögren Syndrome. However, treatment can improve various symptoms and prevent serious complications, both with regard to dryness symptoms and internal organ damage. In medicine, it is difficult to develop a cure for a disease for which the causes are unknown. This is the case for Sjögren Syndrome, whose origin probably involves various factors that are both internal (genetic) and external (infections, toxins, etc.). This scenario currently means it is impossible to cure the disease.
Sjögren Syndrome can be serious, but it is almost never fatal by itself, if the complications are diagnosed early and treated by a specialist in the disease. Patients with Sjögren Syndrome should be carefully monitored to check the development of internal organ involvement, related autoimmune diseases, and other serious complications. In particular, patients should be aware that the incidence of lymphoma (cancer of the lymph nodes) is significantly higher in Sjögren sufferers than the general population.
In the attack on the immune system, the white blood cells that normally fight infections, the lymphocytes, are directed towards the body's fluid-producing glands, mainly the tear and salivary glands. Although it is not known exactly how the damage occurs, the damaged glands can no longer produce tears and saliva, resulting in dryness in the eyes and mouth. When the skin, paranasal sinuses, airways, and vaginal tissues are affected, dryness also occurs in those places.
Systemic autoimmune diseases (SAD) have two essential characteristics. In the first place, in all of these, the body's immune system fails to do its job properly (i.e., defend us from external aggression), and it begins to damage our tissues and cells (autoimmune disease). The second characteristic is that almost all the organs and tissues can be affected by SAD, and it is common for these effects to be multiple and simultaneous (systemic disease). Its main characteristics are, therefore, a lack of knowledge about its aetiology, an effect on multiple (often vital) organs, a complex diagnostic approach and, finally, the use of a wide variety of drugs to treat the disease and its complications.
No. Connective tissue diseases or collagenosis are erroneously assigned to SAD, as this term was coined last century when the autoimmune origin of these diseases was unknown. Connective tissue is what gives tissues their shape and consistency, and takes part in many of their functions; cartilage and fat are examples of connective tissue. There are more than 200 diseases affecting connective tissue, many of which are genetic, like Ehlers-Danlos syndrome, Marfan syndrome, and osteogenesis imperfecta, caused by the incorrect synthesis of collagen, leading to alterations in its structure.
This is a purely statistical or epidemiological definition and therefore encompasses all kinds of diseases, although the vast majority are genetic in origin and particularly affect children. On 1st December, 1999, the first Community Action Plan on Rare Diseases was adopted. This defined rare disease as all those whose prevalence was below 5 cases per 10,000 people in the European Community. Cases of Sjögren Syndrome are higher than this figure, so it cannot be considered a rare disease.
The classification criteria are drawn up by specialists from all over the world who do research on SAD. To be classified as having a SAD, usually the patient has to meet several criteria, but these criteria are problematic because the classifications change over the years. Patients also evolve and the criteria may not be met because initially one type of symptomatology was diagnosed that has nothing to do with that which presents later on. For this reason, continuous monitoring is very important, because the disease or symptoms may change and other diseases may even appear.
Another frequent problem is variability in the results of immunological tests. These may be positive or negative depending on the laboratory they are performed in, and the technique used, so it is important for the doctor to be aware of this variability, which can often modify their diagnostic attitude towards the patient.
Yes. When another disease is associated in the same patient, one process cannot be more important than the other. These clinical situations require SAD experts, because it is often difficult to identify whether there is one or more autoimmune disease, as some have identical clinical symptoms.
For more than 50 years, corticosteroids have been the mainstay of SAD therapy, although immunosuppressant drugs were later added. Over the past decade, biological drugs have been incorporated, which has allowed a better management of patients, with greater effectiveness and fewer side effects. A fundamental goal of the Systemic Autoimmune Diseases Working Group (GEAS) is therefore to change the guidelines that have been applied for years, for others that reduce morbidity. The best examples are the recommendations to use lower and lower doses of corticoids, and for the shortest possible time, and developing robust protocols for the use of new biological therapies.
These are drugs that have specific molecular targets and are based on the exogenous administration of various types of synthetic molecules related to the immune response (antibodies, soluble receptors, cytokines, cytokine antagonists, etc.). There is still a long way to go, starting by defining the correct location of these therapies in the therapeutic strategy of these diseases and their specific indications.
Trusted doctors, used to dealing with Sjögren Syndrome sufferers, provide a guarantee and a certain degree of security, particularly healthcare staff with a comprehensive and systemic perspective. The more cases of SAD they have treated, the better. Due to the way they view a patient as a whole (rather than looking at individual organs), a specialist in internal medicine (or rheumatologist) is better able to care for a patient with Sjögren Syndrome.
Absolutely. Primary care staff must be involved as this is the first step in the patient’s treatment, and they play a key role in the early identification of the disease. Primary care staff are key, and form a crucial part of the medical team in charge of diagnosing and monitoring these patients. They also play an important role in managing vascular risk, the vaccination policy, and the early detection of infection or outbreaks of autoimmune diseases.
Given the systemic nature of Sjögren Syndrome, virtually all specialities may at some point contribute to the ongoing care of SAD patients. It is, therefore, very important to organise patient care into multidisciplinary and multiprofessional teams.
Substantiated information by:
Published: 28 May 2019
Updated: 28 May 2019
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