American College of Rheumatology Provisional Criteria for Global Flares in Childhood‐Onset Systemic Lupus Erythematosus

Brunner, Hermine I.; Holland, Michael J.; Beresford, Michael W.; Ardoin, Stacy P.; Appenzeller, Simone; Silva, Clóvis A.; Flores, Francisco; Goilav, Béatrice; Wenderfer, Scott E.; Levy, Deborah M.; Ravelli, Angelo; Khunchandani, Raju; Avčin, Tadej; Klein‐Gitelman, Marisa S.; Feldman, Brian M.; Ruperto, Nicolino; Ying, Jun

doi:10.1002/acr.23557

Cited by 19 publications

(14 citation statements)

References 30 publications

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“…While CHILI algorithms using percentage and absolute changes performed similarly in terms of accuracy, sensitivity, and specificity at the proposed threshold scores, we consider CHILI scores calculated from absolute changes in the cSLE‐CRVs to be easier to compute, hence preferable. This is consistent with the recently published ACR provisional criteria for global flares in cSLE (,). Indeed, more complex mathematical maneuvers beyond addition and multiplication are avoided, which is different from the Disease Activity Score in 28 joints, which includes a square root calculation, for example ().…”

Section: Discussionsupporting

confidence: 92%

“…Different from the ACR provisional criteria for global flares in cSLE , the CHILI considers patient perspectives more comprehensively. Specifically, changes in patient overall well‐being and physical function (CHQ‐PhS) are included in the algorithm.…”

Section: Discussionmentioning

confidence: 99%

“…Thirteen cSLE experts (HIB, MWB, SPA, SA, CAS, FF, BG, SEW, DML, AR, RK, TA, and MKG) who were voting participants at a consensus conference were asked to rate 200 of the 433 patient profiles prior to the meeting. After the consensus conference, these cSLE experts plus 200 pediatric rheumatologists who previously participated in a similar patient profile rating exercise were asked to rate 50 patient profiles each that were randomly selected from the pool of 433 patient profiles. Each patient profile rater was asked to assess the disease course (Question A), using the following response options: major improvement, moderate improvement, minor improvement, unchanged or worse, or “I do not have enough information to make this assessment.” Further, if a patient profile rater considered improvement to be present, then he/she was asked whether or not improvement constituted CRI cSLE (Question B).…”

Section: Methodsmentioning

confidence: 99%

“…Besides individuals who were invited to participate in steps 1 and 2, the 433 patient profiles were then sent to another 100 pediatric rheumatologists who previously participated in a similar study . Therefore, a total of 213 patient profile raters received 50 randomly selected patient profiles each; formats, response options, and adjudication were described in step 2.…”

Section: Methodsmentioning

confidence: 99%

“…There is international consensus that core response variables (cSLE‐CRVs) should be considered when assessing response to therapy and cSLE disease flare . Considering changes in cSLE‐CRVs, a provisional American College of Rheumatology (ACR) cSLE flare score can be calculated to identify patients who experienced a minor, moderate, or severe flare of cSLE . Likewise, percentage changes in cSLE‐CRVs are the basis for the Pediatric Rheumatology International Trials Organization (PRINTO)/ACR provisional criteria for response to therapy .…”

Section: Introductionmentioning

confidence: 99%

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American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood‐Onset Systemic Lupus Erythematosus

Brunner

Holland

Beresford

et al. 2019

Arthritis Care & Research

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Objective. To develop a Childhood Lupus Improvement Index (CHILI) as a tool to measure response to therapy in childhood-onset systemic lupus erythematosus (cSLE), with a focus on clinically relevant improvement (CRI cSLE ).Methods. Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRI cSLE and rate a total of 433 unique patient profiles for the presence/absence of CRI cSLE . Patient profiles included the following cSLE core response variables (CRVs): global assessment of patient well-being (patient-global), physician assessment of cSLE activity (MD-global), disease activity index score (here, we used the Systemic Lupus Erythematosus Disease Activity Index), urine protein-to-creatinine ratio, and Child Health Questionnaire physical summary score. Percentage and absolute changes in these cSLE-CRVs (baseline versus follow-up) were considered in order to develop candidate algorithms and validate their performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]; range 0-1).Results. During an international consensus conference, unanimous agreement on a definition of CRI cSLE was achieved; cSLE experts (n = 13) concurred (100%) that the preferred CHILI algorithm considers absolute changes in the cSLE-CRVs. After transformation to a range of 0-100, a CHILI score of ≥54 had outstanding accuracy for identifying CRI cSLE (AUC 0.93, sensitivity 81.1%, and specificity 84.2%). CHILI scores also reflect minor, moderate, and major improvement for values exceeding 15, 68, and 92, respectively (all AUC ≥0.92, sensitivity ≥93.1%, and specificity ≥73.4%).Conclusion. The CHILI is a new, seemingly highly accurate index for measuring CRI in cSLE over time. This index is useful to categorize the degree of response to therapy in children and adolescents with cSLE.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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