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. 2019 May;71(5):579-590.
doi: 10.1002/acr.23834.

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

Hermine I Brunner  1 , Michael J Holland  1 , Michael W Beresford  2 , Stacy P Ardoin  3 , Simone Appenzeller  4 , Clovis A Silva  5 , Francisco Flores  1 , Beatrice Goilav  6 , Pinar Ozge Avar Aydin  1 , Scott E Wenderfer  7 , Deborah M Levy  8 , Angelo Ravelli  9 , Raju Khubchandani  10 , Tadej Avcin  11 , Marisa S Klein-Gitelman  12 , Nicolino Ruperto  9 , Brian M Feldman  8 , Jun Ying  13 ; Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group
Collaborators, Affiliations

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

Hermine I Brunner et al. Arthritis Care Res (Hoboken). 2019 May.

Abstract

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 (CRIc SLE ).

Methods: Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRIc SLE and rate a total of 433 unique patient profiles for the presence/absence of CRIc SLE . 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 CRIc SLE 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 CRIc SLE (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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Conflict of interest statement

Michael J. Holland None declared

Michael W. Beresford None declared

Stacy P. Ardoin None declared

Simone Appenzeller None declared

Clovis A. Silva None declared

Francisco Flores None declared

Beatrice Goilav None declared

Pinar Ozge Avar Aydin None declared

Scott E. Wenderfer None declared

Deborah M. Levy None declared

Angelo Ravelli <$10,000: Personal fees (Consultancies & speaking fees) from: AbbVie, BMS, Pfizer, Hoffman LaRoche, Novartis, Centocor, “Francesco Angelini” and Reckitt Benckiser.

Raju Khubchandani None declared

Tadej Avcin <$10,000: AbbVie (consultancies, speaking fees) Octapharma (consultancies, speaking fees) Boehringer Ingelheim (consultancies)

Marisa S. Klein-Gitelman None declared

Brian M. Feldman None declared

Jun Ying None declared

CONFLICT OF INTERESTS

Figures

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References

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