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Arthritis Care Res (Hoboken). 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.

Collaborators (188)

Battagliotti C, Brusco MI, Cuttica R, De Cunto C, Espada G, Farfan M, Garay S, Marcantoni M, Marcela A, Meiorin S, Rama ME, Russo R, Torre Walsh C, Zamparo C, Adib N, Akikusa J, Boros C, Lee SJ, Mckay D, Piper S, Joos R, Bica B, Campos L, Cavalcanti A, do Prado R, Donner-Maliki A, Fernandes T, Fonseca A, Gasparello de Almeida R, Guariento A, Gusman C, Jusan Fiorot F, Knupp Oliveira S, Len C, Arruda Campos LM, Machado S, Marques L, Martins de Carvalho L, Moulin R, Pedroso S, Pileggi G, Romanelli PRS, Saad-Magalhaes C, Sakamoto A, Santos MC, Silva MF, Spelling P, Sztajnbok S, Terreri M, Cabral D, Chédeville G, Ellsworth J, Huber A, Tucker L, Houghton K, Borzutzky A, Ladino M, Norambuena X, Eraso R, Mosquera A, Velasquez M, Harjacek M, Jelusic M, Coto Hermosilla C, Dolezalova P, Nielsen S, Tineo C, Alegria M, Dressler F, Foell D, Ganser G, Hinze C, Hufnagel M, Lutz T, Trauzeddel R, Boiu S, Trachana M, Tsitsami E, Cifuentes M, Orbán I, Aggarwal A, Sawhney S, Butbul Aviel Y, Cimaz R, Maggio MC, Rumba-Rozenfelde I, Hashad S, Lim SC, Abud C, Burgos-Vargas R, Carreño-Manjarrez R, Enciso Pelaez S, Hernandez-Huirache H, Maldonado Velázquez R, Orozco J, Rodriguez-Lozano AL, Rojas Pacheco OE, Suárez Larios LM, Vega G, Ramírez Miramontes JV, Ruíz Lopez IK, Kamphuis S, Schonenberg-Meinema D, Concannon A, Yan J, Jarquin Jaime M, Al Abrawi S, Vega C, Lopez-Benitez J, Ibáñez Estrella A, Miraval T, Dans L, Kimseng KJ, Opoka-Winiarska V, Rutkowska-Sak L, Smolewska E, Conde M, Guedes M, Del Valle E, Quintero-Del Rio A, Ailioaie C, Sparchez M, Alekseeva E, Keltsev V, Al-Mayouf S, Asiri A, Suwairi W, Susic G, Vijatov-Djuric G, Ang E, Arkachaisri T, Boteanu AL, Lopez-Robledillo JC, Medrano San Ildefonso M, Modesto C, Calvo I, Sotoca-Fernandez J, Bolt I, Vilaiyuk S, Al-Abadi E, Baildam E, Fotis L, Pain C, Pilkington C, Abulaban K, Barbar-Smiley F, Binstadt B, Bohnsack J, Boneparth A, Brown D, Chira P, Cron R, Dedeoglu F, Eberhard A, Gedalia A, Grom A, Henrickson M, Hom C, Huggins J, Jerath R, Jones J, Jung L, Kingsbury D, Lai J, Lovell D, Nanda K, Nocton J, Olson J, O'Neil K, Onel K, Punaro L, Reiff A, Rouster-Stevens K, Ruth N, Schikler K, Murphy Schmidt K, Schulert G, Shaham B, Singer N, Smith J, Sundel R, Syverson G, Vega-Fernandez P, Vehe R, Wagner-Weiner L, Cameto J, Jurado R, Maldonado I.

Author information

1
University of Cincinnati and Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
2
Institute of Translational Medicine and Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
3
Ohio State University, Nationwide Children's Hospital, and Wexner Medical Center, Columbus, Ohio.
4
University of Campinas, Campinas, Brazil.
5
Children's Institute, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, São Paulo, Brazil.
6
Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York.
7
Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
8
University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada.
9
Clinica Pediatrica e Reumatologia, Istituto Giannina Gaslini, PRINTO, Genoa, Italy.
10
Jaslok Hospital Mumbai, India.
11
University Children's Hospital, University Medical Centre, Ljubljana, Slovenia.
12
Northwestern University Feinberg School of Medicine and Ann and Robert Lurie Children's Hospital of Chicago, Chicago, Illinois.
13
University of Cincinnati, Cincinnati, Ohio.

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.

PMID:
30680946
PMCID:
PMC6483875
[Available on 2020-05-01]
DOI:
10.1002/acr.23834

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