The new classification criteria reflect current thinking about Lupus and provide an improved foundation for research.
Systemic lupus erythematosus (often called Lupus or shortened to SLE) is an autoimmune disease. It typically affects women between the ages of 15 and 45, but can also start in younger children, later in life, and also in men. Lupus causes immune cells in the body to become hyperactive and produce autoantibodies. Symptoms vary, but people with Lupus are often very tired, have joint pain, and their skin may be sensitive to sunlight. Inner organs such as the kidneys or brain may also be affected.
Classification criteria are used to scientifically define who has a disease. They are particularly important for planning clinical trials for new treatments, but also help doctors and medical students to recognise the disease.
What did the authors hope to find?
The authors wanted to better understand Lupus, and to develop new tools to help classify the disease.
Who was studied?
The study looked at 1,925 people with Lupus and 1,301 people with other types of rheumatic diseases in 21 clinics from across the world. Data from more than 10,000 people with Lupus from published papers and studies were also included.
How was the study conducted?
This was a complicated study with several parts. To begin, information about antinuclear antibodies (often shortened to ANA) was collected from a literature review. Signs and symptoms of Lupus were collected from surveys of Lupus experts and people with the disease. From this, the authors created a list of possible classification criteria, which were then examined and analysed by a group of 17 experts. Finally, the criteria were tested in over 2,000 people with and without Lupus. In this last part, everyone’s Lupus status had been confirmed in three different ways to ensure that the groups were correct.
What are the main classification criteria?
Almost all people with Lupus test positive for ANA. Because of this, the new criteria now use ANA-positivity as the first step for classifying Lupus. People who test positive for ANA can be classified as having Lupus when they also score 10 points from a list of 21 other symptoms and clinical tests including fever, blood count changes, inflammation of the pericardium or pleura, brain dysfunction, joint involvement or kidney involvement. Importantly, the other symptoms each have a different (or weighted) score from 2–10, depending on how important they are.
Are these findings new?
Only non-infectious fever is a new item in the classification criteria. What is new is the position of ANA as an entry criterion and the weighting placed on the other symptoms. The rule that symptoms are only counted if not better explained by another disease (or drug) is also new.
What are the limitations of the study?
Despite the worldwide cooperation in the project and the large numbers of people taking part, involvement around the globe was not consistent. Because of this, the criteria will need to be tested in less represented groups.
What do the authors plan on doing with this information?
The authors hope that the new criteria will now be discussed and tested worldwide in more studies.
What does this mean for me?
These criteria will mainly be used in clinical trials to ensure that people with other types of autoimmune conditions are not included in Lupus trials and scientific studies. The information from this project might also be used by doctors to help recognise symptoms and diagnose Lupus. However, classification is not the same as an
individual diagnosis, which includes much more information and may not be fully aligned with classification in all people.
If you think that you might have Lupus, you should speak to your doctor.
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Date prepared: August 2019
Summary based on research article published on: 5 August 2019
From: Aringer M, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151–9. doi:10.1136/annrheumdis-2018-214819
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