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The American College of Rheumatology Provisional criteria for clinically relevant improvement in children & adolescents with childhood-onset systemic lupus erythematosus


Brunner HI1, Holland MJ1, Beresford MW2,3, Ardoin SP4, Appenzeller S5, Silva CA6, Flores F7, Goilav B8, Avar Aydin PO1, Wenderfer SE9, Levy DM10, Ravelli A11, Khubchandani R12, Avcin T13, Klein-Gitelman MS14, Ruperto N11, Feldman BM10, Ying J15; Paediatric Rheumatology International Trial Organisation & the Pediatric Rheumatology Collaborative Study Group. Arthritis Care Res (Hoboken). 2019 Jan 25. doi: 10.1002/acr.23834.

Author Information

1 Department of Pediatrics, University of Cincinnati College of Medicine and Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

2 Institute of Translational Medicine, University of Liverpool, Liverpool, UK.

3 Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK.

4 Department of Pediatrics and Internal Medicine, Ohio State University, Division of Rheumatology, Nationwide Children's Hospital and Wexner Medical Center, Columbus, OH.

5 Rheumatology Unit, School of Medical Science, University of Campinas, Campinas, Brazil.

6 Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina da Universidade de São Paulo, Brazil.

7 Department of Pediatrics, University of Cincinnati College of Medicine and Division of Nephrology & Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

8 The Children's Hospital at Montefiore, Division of Nephrology and Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY.

9 Department of Pediatrics, Baylor College of Medicine, Renal Section, Texas Children's Hospital Houston, TX.

10 Department of Pediatrics, University of Toronto and Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada.

11 Istituto G. Gaslini, Clinica Pediatrica e Reumatologia, PRINTO, Genoa, Italy.

12 Pediatric Rheumatology, Jaslok Hospital Mumbai, India.

13 Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Slovenia.

14 Department of Pediatrics, Northwestern University, Feinberg School of Medicine and Division of Rheumatology, Ann and Robert Lurie Children's Hospital of Chicago, Chicago, IL.

15 Department of Environmental Health Sciences, University of Cincinnati, Cincinnati, OH.



To develop a Childhood Lupus Improvement Index (CHILI) as a tool to measure response to therapy in childhood-onset systemic lupus erythematosus (cSLE), with focus on clinically relevant improvement (CRIc SLE ).


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 cSLE core response variables [cSLE-CRVs: global assessment of patient well-being (Patient-global), physician assessment of cSLE activity (MD-global), disease activity index score (here: Systemic Lupus Erythematosus Disease Activity Index), urine protein-to-creatinine ratio (UPCR), Child Health Questionnaire physical summary score (CHQ-PhS)]. Percentage and absolute changes of these cSLE-CRVs (baseline vs. follow-up) were considered to develop candidate algorithms and validate their performance [sensitivity, specificity, area under the receiver operating characteristic curve (AUC; range: 0 -1)].


Using an international consensus conference, unanimous agreement on a definition of CRIc SLE was achieved; cSLE expects (n=13) concurred (100%) that the preferred CHILI algorithm considers absolute changes of the cSLE-CRVs. After transformation to range from 0 -100, a CHILI score of >54 had outstanding accuracy for identifying CRIc SLE (AUC=0.93; sensitivity=81.1%; specificity=84.2%); CHILI scores also reflect minor, moderate and major improvement for values exceeding 15, 68 and 92 (all: AUC > 0.92, sensitivity: >93.1%; specificity: >73.4%).


The CHILI is a new, seemingly highly accurate index for measuring clinically important improvement in cSLEover time. This index is useful to categorize the degree of response to therapy in children and adolescent with cSLE.