Faculty Collaboration Database

American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus. Arthritis Care Res (Hoboken) 2019 05;71(5):579-590

Date

01/27/2019

Pubmed ID

30680946

Pubmed Central ID

PMC6483875

DOI

10.1002/acr.23834

Scopus ID

2-s2.0-85064810180   1 Citation

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 (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.

Author List

Brunner HI, Holland MJ, Beresford MW, Ardoin SP, Appenzeller S, Silva CA, Flores F, Goilav B, Avar Aydin PO, Wenderfer SE, Levy DM, Ravelli A, Khubchandani R, Avcin T, Klein-Gitelman MS, Ruperto N, Feldman BM, Ying J, Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group

Author

Judyann C. Olson MD Associate Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Algorithms
Antirheumatic Agents
Child
Delphi Technique
Humans
Lupus Erythematosus, Systemic
Severity of Illness Index