Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Preliminary Criteria for Global Disease Flares in Juvenile Systemic Lupus Erythematosus (jSLE).

Mina8,  Rina, Beresford12,  Michael, Eberhard13,  B. Anne, Giannini3,  Edward H., Levy15,  Deborah M., Pilkington10,  Clarissa, Punaro14,  Marilynn G.

BC Childrens Hospital, Vancouver, BC, Canada
Great Ormond Street and University College London Hospitals
IRCCS G Gaslini
Royal Liverpool Children's Hospital
Schneider Children's Hospital, New Hyde Park, NY
Texas Scottish Rite Hospital for Children, Dallas, TX
The Hospital for Sick Children, Toronto, ON, Canada
University of Cincinnati
Childrens Hosp LA MS60, Los Angeles, CA
Childrens Hosp Med Ctr, Cincinnati, OH
Childrens Mem Hosp/NW Univ, Chicago, IL
Cincinnati Children's Hospital
Cincinnati Children's Hospital, Cincinnati, OH
Cincinnati Children's Hospital Medical Ctr, Cincinnati, OH
Cincinnati Children's Med Ctr, Cincinnati, OH
Duke Univ Medical Center, Durham, NC


Widely accepted valid, reliable and feasible flare criteria to assess the effects of new medications and the benefits of current treatments in jSLE are lacking.


Develop accurate flare criteria for jSLE by employing consensus formation methodology and statistical approaches.


As part of a consensus formation process, an international group of pediatric rheumatology experts (n=138) were randomized to rate a total of 400 different patient profiles that were abstracted from prospective jSLE cohorts. jSLE flare descriptors deemed important, as per previous Delphi surveys (patient global assessment, physician global assessment, disease activity score, health status, proteinuria, anti-dsDNA antibody titer, ESR, complement C3 and C4), were considered when generating candidate flare algorithms, using various percentage changes of the flare descriptors, examination of flare criteria used in adult SLE, multinomial logistic regression, and Classification and Regression Tree (CART). For each candidate flare algorithm overall accuracy, sensitivity and specificity was calculated, using the consensus of the profile raters (minor flare, moderate flare, major flare, no flare) as criterion standard. An international consensus conference was held to rank the generated candidate flare algorithms following the process previously suggested by the ACR Criteria Subcommittee (consensus level 75%).


A total of 2,995 expert ratings were available for analysis (survey response rate 70%). Consensus was reached that jSLE flare criteria derived by multinomial logistic regression or CART analysis are most suitable to measure jSLE flares. The highest ranked such candidate flare criteria consider absolute changes in the score of a disease activity index (SLEDAI or BILAG), the physician global assessment (PGA), spot urine protein/creatinine (P/C) ratio, and ESR when calculating a jSLE flare score. This score is then used to determine the presence of a global flare and to discriminate among categories of flare severity (minor, moderate and major). The highest ranked preliminary jSLE flare criteria have excellent to outstanding accuracy as measured by the area under the Receiver Operating Characteristic Curve (AUC) (see Table).

 Accuracy (AUC)
Preliminary Criteria for Global Flares(All algorithms use change in variables between follow up-baseline visit)Minor FlareModerate FlareMajor Flare
A. 0.5 × SLEDAI + 0.45 × P/C ratio + 0.5 × PGA + 0.02 × ESR0.900.921.00
B. 0.4 × BILAG + 0.65 × P/C ratio + 0.5 × PGA + 0.02 × ESR0.890.920.96
C. 0.4 × SLEDAI + 0.33 × P/C ratio + 0.6 × PGA0.870.921.00
D. 0.4 × BILAG + 0.55 × P/C ratio + 0.5 × PGA0.880.920.95
E. CART analysis (patient is categorized by highest flare level)0.890.940.99
Major Flare: SLEDAI >= 10 OR PGA >= 6   
Moderate Flare: 7 <= SLEDAI <= 9 OR P/C ratio >= 2.3 OR ESR >= 13   
Minor Flare: 3 <= SLEDAI <= 6 OR 2 <= PGA < 6 OR 0.7 < P/C ratio <= 2.2   


Consensus has been reached on the preliminary criteria for global flares in jSLE. Prospective validation of these criteria is needed to assess their usefulness as outcomes for clinical trials and other research settings.

To cite this abstract, please use the following information:
Mina, Rina, Beresford, Michael, Eberhard, B. Anne, Giannini, Edward H., Levy, Deborah M., Pilkington, Clarissa, et al; Preliminary Criteria for Global Disease Flares in Juvenile Systemic Lupus Erythematosus (jSLE). [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1688
DOI: 10.1002/art.29453

Abstract Supplement

Meeting Menu