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ACR provisional criteria tracks modest improvement in childhood SLE
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Researchers have developed a novel, seemingly highly accurate index for measuring improvement in juvenile systemic lupus erythematosus over time, representing an international consensus on a definition of “clinically relevant improvement” among children and adolescents with the disease, according to recent findings in Arthritis Care & Research.
“We have previously shown, albeit in a rather small dataset, that the PRINTO/ACR Provisional Criteria for Response to Therapy and, to a lesser extent, the Systemic Lupus Responder Index are both very well-suited to capture major improvement of [childhood] SLE; however, both the PRINTO/ACR Provisional Criteria of Response to Therapy and the Systemic Lupus Responder Index appeared less apt to identify patients who experienced moderate or minor improvement of [childhood] SLE,” Hermine I. Brunner, MD, MSc, of the Cincinnati Children’s Hospital Medical Center, and colleagues wrote.
The researchers added, “At present, there are no generally accepted criteria or algorithms to measure various degrees of improvement with [childhood] SLE, and consensus is lacking of what constitutes clinically relevant improvement in children and adolescents with [childhood] SLE.”
To develop a tool to measure treatment response in patients with childhood onset SLE, referred to by the researchers as the Childhood Lupus Improvement Index (CHILI), Brunner and colleagues conducted a Delphi survey involving a total of 13 experts in childhood SLE and 200 pediatric rheumatologists. These participants were invited to come to a consensus on a definition of clinically relevant improvement in childhood-onset SLE, and to rate 433 unique patient profiles for the disease’s presence or absence.
These patient profiles included the following childhood-SLE core response variables: Global assessment of patient well being, physician assessment of disease activity, Systemic Lupus Erythematosus Disease Activity Index, urine protein to creatinine ratio and Child Health Questionnaire physical summary score. The researchers used the percentage and absolute changes of these core response variables from baseline to develop candidate algorithms and validate their performance.
According to Brunner and colleagues, the 13 childhood SLE experts came to a 100% consensus that the preferred CHILI algorithm would consider absolute changes of childhood SLE’s core response variables. After transformation to a range of 0 to 100, a CHILI score of greater than 54 had an outstanding accuracy for identifying clinically relevant improvement, with an area under curve of 0.93, a sensitivity of 81.1% and a specificity of 84.2%.
The researchers also noted that CHILI scores additionally reflected minor, moderate and major improvement for values exceeding 15, 68 and 92, with an area under curve greater than 0.92, a sensitivity greater than 93.1% and a specificity greater than 73.4% for all.
“This Provisional CHILI instrument can be used to help identify children with [childhood] SLE who have experienced a clinically relevant improvement and to categorize the degree of improvement as minor, moderate or major,” Brunner and colleagues wrote. “However, additional testing in independent datasets is required to confirm the performance characteristics of the CHILI when used in [childhood] SLE.” – by Jason Laday
Disclosure: Brunner reports consulting fees from AbbVie, Ablynx, Amgen, AstraZeneca, Baxalta Biosimilars, Biogen Idec, Boehringer Ingelheim, Bristol-Myers Squibb, Celgene, Eli-Lilly, EMD Serono, Gilead Sciences, Janssen, MedImmune, Novartis, Pfizer, R-Pharm, Roche, Servier and Takeda; speaking fees from Genentech and Novartis; and grant support from Pfizer and Bristol-Myers Squibb. Please see the study for all other authors’ relevant financial disclosures.
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