The committee agreed not to count a feature when it is more likely due to another condition and to create weighted criteria. Example: Such manifesttations as lupus nephritis carry a higher score than less common skin features. If the score is 10 or higher, the patient is classified as having SLE.
The committee wanted to reduce bias and circular reasoning, which could affect the criteria’s validity and viability, said co-chair Sindhu Johnson, MD, PhD, director of the scleroderma program at the University of Toronto.3 In the past, the same experts derived the criteria and then submitted cases to test them, she said. Separate panels, including rheumatologists, dermatologists, nephrologists and pediatricians, were recruited for each phase. SLE patients were surveyed to find out what symptoms they experienced early in their disease.
The experts nominated criteria that distinguish early from established disease. “We also asked them to think more broadly: Could these criteria also be used for juvenile-onset SLE? Or to identify established disease, and also early disease?” said Dr. Johnson. They found that manifestations of both early and established SLE, such as autoantibodies, specific renal features and skin manifestations, are also seen in mimickers. In addition, 85% said they would positively classify a patient if renal pathology alone showed lupus nephritis.
Looking at data from early SLE cohorts, “it appears that a number of classical lupus manifestations do distinguish SLE from mimickers, notably kidney disease, oral ulcers, pleuritis and alopecia,” she said.4 An interesting new finding was that non-classical, less specific manifestations, such as arthralgia, fever and fatigue, also distinguish early SLE from mimickers. “Finally, we looked to the patients. They also reported arthralgia, fever and fatigue as manifestations they experienced early in their disease.”5
A panel of SLE experts reviewed the criteria in development. Their recommendations included developing precise definitions for each criterion and clustering criteria related to the same organ systems into domains or buckets. Both recommendations were validated by the committee and applied.
“Because they are interrelated, it would be inappropriate to doubly count criteria within a domain,” she added. After ranking and scoring by a different expert panel, the criteria were tested in a derivation cohort and found to have a sensitivity of 98% and a specificity of 97%—higher than both the 1997 ACR and 2012 SLICC criteria, she said. The criteria will now be validated against a large cohort.
The development team’s goal is for the criteria to include a mobile app and online tool that define each criterion and add up the patient’s score, said Karen Costenbader, MD, director of the lupus program at Brigham and Women’s Hospital in Boston. She showed the audience how to score several patient cases. Despite their many strengths, these criteria have some limitations, so discussion is welcome, she said.
One thing I don’t like in this criteria ….that is it is mandatory to have positive ANA testing to apply the criteria….this would result in many false negative cases on applying this criteria….. because all of us know that some SLE cases have negative ANA and DNA but they have consumed complement C3 and C4 …so according to the SLICC classification criteria they are having SLE but according to the New criteria they aren’t….!!!!
I agree. And about the patients ANA negative, but anti-SSA/Ro positive? And, these new criteria are too sensive. Everything is lupus?
In1995 I was diagnosed with autoimmune hepatitis following years of pain meds and neck surgeries . I was told after blood work I had positive ANA, negative RA, and a gamete of positive inflammation and other test hoeing autoimmune disease. The Rheumatologist walk in to my hospital room and said I had a classic butterfly rash on my face, blistering in mouth and nose, horrible headaches, but still working. So I went on about 6 months of prednisone and taken off any meds that process through my liver. I got better. I was told my trigger was the trigger of my first flare. Nothing but fatigue and headaches, occasional achy joints. So I rarely went to rheumatologist . 2 years ago I had anaphylactic shock from peanuts, my disease raised its ugly head. Now my mom has had lupus since she was twenty five, discoid type. My youngest daughter diagnosed with SLE at age nine, after very strange lumps on her tendons. She also had kidney issues, . So three women in my family lupus. My currrnt flare has been raging for coming up on two years. Horrible, could not turn over in bed by my self from pain let alone walk. I can walk some now but not for long, cannot stand in lines, can’t bath myself, I have horrible chest pain from pleurisy and lupus pneumonitis. I have trouble swallowing. My joints are hurting all the time. I am now on oxygen. I have two brain masses, and several lumps or masses growing on my groin and abdominal area. My lymph nodes are huge and some are painless while others mainly my temporal nodes are painful. Last night I awoke with vomit on me choking on it and coughing for hours. I am on lots of meds but progress is slow. Last blood work Ana negative.. WTF… my Dr says it doesn’t matter it is Lupus. New Rheumatologist lets do more test, lupus always had positive ANA and RA.. ugh no guys I have Lupus and I am sick. I aspirated last night, I could have died in my sleep easily and now need to worry about..go by the symptom list it seems to be very accurate wether you have one initial episode or many. Hang in and find some Dr that listen to your symptoms, write them down. Good luck