People with evidence of systemic disease at the time of diagnosis have reduced long-term survival compared to people with simple sicca or dryness symptoms.
Sjögren syndrome is an autoimmune disease where the body’s immune system attacks glands that produce fluids, for example the lacrimal and salivary glands. The disease mainly affects women between the ages of 30 and 50. People with Sjögren syndrome may have dry and sore eyes and a dry or sticky mouth and throat. These symptoms are known as sicca (dryness) symptoms. The dryness may also affect vaginal lubrication in women, making sex uncomfortable. Severe cases of Sjögren syndrome can attack internal organs (the lungs, kidneys or the nervous system). This is known as systemic disease. Because Sjögren syndrome is a complex disease with many possible symptoms, it can be difficult to diagnose.
WHAT DID THE AUTHORS HOPE TO FIND?
The authors wanted to see whether there was a link between having a systemic form of Sjögren syndrome and how long people survive for after they are diagnosed. They hoped that being able to identify people with a worse prognosis (outlook) early on in the disease could help doctors to manage them more closely, which might in turn mean that the people survive longer. They also wanted to see how well a disease activity scoring system called EULAR-SS Disease Activity Index (shortened to ESSDAI) could predict which people would die.
WHO WAS STUDIED?
The study looked at 1,045 people with Sjögren syndrome from 20 clinics across Spain. Of the people included, 94% were women, and the average age at diagnosis was 54 years. Almost all of the people had sicca symptoms such as dry mouth or dry eyes. Many also had auto-antibodies in their blood, which can be used as markers of inflammation and the presence of systemic disease.
HOW WAS THE STUDY CONDUCTED?
This was a retrospective observational study, which means that the authors set up a database to collect information on people diagnosed with Sjögren syndrome. There was no interventional treatment given. The authors collected clinical and laboratory information which was then analysed to try to make links between different variables.
WHAT WERE THE MAIN FINDINGS OF THE STUDY?
Overall, the results did not show a significant difference in the ESSDAI score at diagnosis between people who died and those who survived. However, the authors did find that there were some factors that put people at a higher risk of dying within 10 years. These included being male, having very severe disease or disease that affected the lungs, or having more than one marker in the blood at the time of diagnosis. People with systemic activity at the time of diagnosis were at higher risk of developing lymphoma and have worse prognosis than people without systemic activity early in their disease.
ARE THESE FINDINGS NEW?
Until now, all studies looking at the factors that increase the risk of dying in people with Sjögren syndrome have focused on people with no systemic disease. This study is the first to look at a potential link between systemic activity and survival in people with Sjögren syndrome.
HOW RELIABLE ARE THE FINDINGS?
There are some limitations in the study. Most people studied had a very low level of systemic disease at the time of diagnosis. There was therefore only a very small number of patients (less than 1%) with some systemic involvement (for example, in their kidneys or muscles). This does limit the strength of the findings.
WHAT DO THE AUTHORS PLAN ON DOING WITH THIS INFORMATION?
A EULAR task group is working on recommendations for the treatment of certain types of systemic Sjögren syndrome. There is also a database being developed that will collect information globally, and this will hopefully provide more information that will be useful in treating and managing people with Sjögren syndrome.
WHAT DOES THIS MEAN FOR ME?
It is important to understand that having a risk factor does not mean you will definitely die from your disease. These are statistics that doctors use to try to predict outcomes. If you are male, have more severe disease, disease that affects your lungs, or a certain pattern of markers (auto-antibodies) in your blood, your doctor may want to see you more often (every 3 to 6 months) to make sure that you are managed closely and receive appropriate treatment. Other people who have simple dryness symptoms may only need to be seen by their doctor once a year. If you have any new or unexpected symptoms, you should report them to your doctor.
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Date prepared: February 2016
Summary based on research article published on: 28 November 2014
From: Brito-Zerón, P. et al. Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis 2016;75:348–55. doi:10.1136/annrheumdis-2014-206418
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