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

the Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group

Research output: Contribution to journalArticle

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

Original languageEnglish (US)
Pages (from-to)579-590
Number of pages12
JournalArthritis Care and Research
Volume71
Issue number5
DOIs
StatePublished - May 2019

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Systemic Lupus Erythematosus
Area Under Curve
Sensitivity and Specificity
Nephrology
Rheumatology
ROC Curve
Creatinine
Urine
Pediatrics
Physicians
Therapeutics

ASJC Scopus subject areas

  • Rheumatology

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American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus. / the Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group.

In: Arthritis Care and Research, Vol. 71, No. 5, 05.2019, p. 579-590.

Research output: Contribution to journalArticle

the Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group 2019, 'American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus', Arthritis Care and Research, vol. 71, no. 5, pp. 579-590. https://doi.org/10.1002/acr.23834
the Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group. / American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus. In: Arthritis Care and Research. 2019 ; Vol. 71, No. 5. pp. 579-590.
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title = "American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus",
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 c SLE ). Methods: Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRI c SLE and rate a total of 433 unique patient profiles for the presence/absence of CRI c 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 CRI c 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 CRI c 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.",
author = "{the Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group} and Brunner, {Hermine I.} and Holland, {Michael J.} and Beresford, {Michael W.} and Ardoin, {Stacy P.} and Simone Appenzeller and Silva, {Clovis A.} and Francisco Flores and Beatrice Goilav and {Avar Aydin}, {Pinar Ozge} and Wenderfer, {Scott E.} and Levy, {Deborah M.} and Angelo Ravelli and Raju Khubchandani and Tadej Avcin and Klein-Gitelman, {Marisa S.} and Nicolino Ruperto and Feldman, {Brian M.} and Jun Ying and Cristina Battagliotti and Brusco, {Maria Isabel} and Rub{\'e}n Cuttica and {De Cunto}, Carmen and Graciela Espada and Maximiliano Farfan and Stella Garay and Maria Marcantoni and Alvarez Marcela and Silvia Meiorin and Rama, {Maria Elena} and Ricardo Russo and {Torre Walsh}, Carolina and Celso Zamparo and Navid Adib and Jonathan Akikusa and Christina Boros and Lee, {Senq J.} and Damien Mckay and Susan Piper and Rik Joos and Blanca Bica and Leonardo Campos and Andr{\'e} Cavalcanti and {do Prado}, Rogerio and Amanda Donner-Maliki and Taciana Fernandes and Adriana Fonseca and {Gasparello de Almeida}, Rozana and Andressa Guariento and Catherine Gusman and Jerath, {Rita S}",
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TY - JOUR

T1 - American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus

AU - the Paediatric Rheumatology International Trial Organisation and Pediatric Rheumatology Collaborative Study Group

AU - Brunner, Hermine I.

AU - Holland, Michael J.

AU - Beresford, Michael W.

AU - Ardoin, Stacy P.

AU - Appenzeller, Simone

AU - Silva, Clovis A.

AU - Flores, Francisco

AU - Goilav, Beatrice

AU - Avar Aydin, Pinar Ozge

AU - Wenderfer, Scott E.

AU - Levy, Deborah M.

AU - Ravelli, Angelo

AU - Khubchandani, Raju

AU - Avcin, Tadej

AU - Klein-Gitelman, Marisa S.

AU - Ruperto, Nicolino

AU - Feldman, Brian M.

AU - Ying, Jun

AU - Battagliotti, Cristina

AU - Brusco, Maria Isabel

AU - Cuttica, Rubén

AU - De Cunto, Carmen

AU - Espada, Graciela

AU - Farfan, Maximiliano

AU - Garay, Stella

AU - Marcantoni, Maria

AU - Marcela, Alvarez

AU - Meiorin, Silvia

AU - Rama, Maria Elena

AU - Russo, Ricardo

AU - Torre Walsh, Carolina

AU - Zamparo, Celso

AU - Adib, Navid

AU - Akikusa, Jonathan

AU - Boros, Christina

AU - Lee, Senq J.

AU - Mckay, Damien

AU - Piper, Susan

AU - Joos, Rik

AU - Bica, Blanca

AU - Campos, Leonardo

AU - Cavalcanti, André

AU - do Prado, Rogerio

AU - Donner-Maliki, Amanda

AU - Fernandes, Taciana

AU - Fonseca, Adriana

AU - Gasparello de Almeida, Rozana

AU - Guariento, Andressa

AU - Gusman, Catherine

AU - Jerath, Rita S

PY - 2019/5

Y1 - 2019/5

N2 - 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 c SLE ). Methods: Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRI c SLE and rate a total of 433 unique patient profiles for the presence/absence of CRI c 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 CRI c 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 CRI c 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.

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