{"id":594,"date":"2016-02-20T15:26:50","date_gmt":"2016-02-20T15:26:50","guid":{"rendered":"http:\/\/promotions.bmj.com\/ardsummaries\/?p=594"},"modified":"2016-02-20T15:26:50","modified_gmt":"2016-02-20T15:26:50","slug":"systemic-disease-at-diagnosis-predicts-earlier-death-in-sjogren-syndome","status":"publish","type":"post","link":"https:\/\/blogs.bmj.com\/rheumsummaries\/2016\/02\/20\/systemic-disease-at-diagnosis-predicts-earlier-death-in-sjogren-syndome\/","title":{"rendered":"Systemic disease at diagnosis predicts earlier death in Sj\u00f6gren syndome"},"content":{"rendered":"<p>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.<\/p>\n<p><strong> INTRODUCTION<\/strong><br \/>\nSj\u00f6gren syndrome is an autoimmune disease where the body\u2019s 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 Sj\u00f6gren 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 Sj\u00f6gren syndrome can attack internal organs (the lungs, kidneys or the nervous system). This is known as systemic disease. Because Sj\u00f6gren syndrome is a complex disease with many possible symptoms, it can be difficult to diagnose.<\/p>\n<p><!--more--><\/p>\n<p><strong>WHAT DID THE AUTHORS HOPE TO FIND?<\/strong><br \/>\nThe authors wanted to see whether there was a link between having a systemic form of Sj\u00f6gren 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.<\/p>\n<p><strong>WHO WAS STUDIED?<\/strong><br \/>\nThe study looked at 1,045 people with Sj\u00f6gren 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.<\/p>\n<p><strong>HOW WAS THE STUDY CONDUCTED?<\/strong><br \/>\nThis was a retrospective observational study, which means that the authors set up a database to collect information on people diagnosed with Sj\u00f6gren 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.<\/p>\n<p><strong>WHAT WERE THE MAIN FINDINGS OF THE STUDY?<\/strong><br \/>\nOverall, 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.<\/p>\n<p><strong>ARE THESE FINDINGS NEW?<\/strong><br \/>\nUntil now, all studies looking at the factors that increase the risk of dying in people with Sj\u00f6gren 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 Sj\u00f6gren syndrome.<\/p>\n<p><strong>HOW RELIABLE ARE THE FINDINGS?<\/strong><br \/>\nThere 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.<\/p>\n<p><strong>WHAT DO THE AUTHORS PLAN ON DOING WITH THIS INFORMATION?<\/strong><br \/>\nA EULAR task group is working on recommendations for the treatment of certain types of systemic Sj\u00f6gren 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 Sj\u00f6gren syndrome.<\/p>\n<p><strong>WHAT DOES THIS MEAN FOR ME?<\/strong><br \/>\nIt 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.<\/p>\n<p><strong>Disclaimer:<\/strong> This is a summary of a scientific article written by a medical professional (\u201cthe Original Article\u201d). The Summary is written to assist non medically trained readers to understand general points of the Original Article. It is supplied \u201cas is\u201d without any warranty. You should note that the Original Article (and Summary) may not be fully relevant nor accurate as medical science is constantly changing and errors can occur. It is therefore very important that readers not rely on the content in the Summary and consult their medical professionals for all aspects of their health care and only rely on the Summary if directed to do so by their medical professional. Please view our full <a href=\"http:\/\/www.bmj.com\/company\/legalinformation\/\" target=\"_blank\" rel=\"noopener noreferrer\">Website Terms and Conditions.<\/a><\/p>\n<p>Date prepared: February 2016<\/p>\n<p>Summary based on research article published on: 28 November 2014<\/p>\n<p><strong>From:<\/strong> Brito-Zer\u00f3n, P. et al. Systemic activity and mortality in primary Sj\u00f6gren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis 2016;75:348\u201355. doi:10.1136\/annrheumdis-2014-206418<\/p>\n<p>Copyright \u00a9 2016 BMJ Publishing Group Ltd &amp; European League Against Rheumatism. Medical professionals may print copies for their and their patients and students non commercial use. Other individuals may print a single copy for their personal, non commercial use. For other uses please contact our <a href=\"http:\/\/www.bmj.com\/company\/products-services\/rights-and-licensing\/\" target=\"_blank\" rel=\"noopener noreferrer\">Rights and Licensing <\/a>Team.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>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. INTRODUCTION Sj\u00f6gren syndrome is an autoimmune disease where the body\u2019s immune system attacks glands that produce fluids, for example the lacrimal and salivary glands. The disease mainly affects women between the [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/blogs.bmj.com\/rheumsummaries\/2016\/02\/20\/systemic-disease-at-diagnosis-predicts-earlier-death-in-sjogren-syndome\/\">Read More&#8230;<\/a><\/p>\n","protected":false},"author":384,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[21],"tags":[],"class_list":["post-594","post","type-post","status-publish","format-standard","hentry","category-sjogren-syndome"],"_links":{"self":[{"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/posts\/594","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/users\/384"}],"replies":[{"embeddable":true,"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/comments?post=594"}],"version-history":[{"count":0,"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/posts\/594\/revisions"}],"wp:attachment":[{"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/media?parent=594"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/categories?post=594"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blogs.bmj.com\/rheumsummaries\/wp-json\/wp\/v2\/tags?post=594"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}