American College of Rheumatology Provisional Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus

Hermine I. Brunner, Michael Holland, Michael W. Beresford, Stacy P. Ardoin, Simone Appenzeller, Clovis A. Silva, Francisco Flores, Beatrice Goilav, Scott E. Wenderfer, Deborah M. Levy, Angelo Ravelli, Raju Khunchandani, Tadej Avcin, Marisa S. Klein-Gitelman, Brian M. Feldman, Nicolino Ruperto, Jun Ying

Research output: Contribution to journalArticle

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Abstract

Objective: To validate the preliminary criteria of global flare for childhood-onset SLE (cSLE). Methods: Pediatricians experienced in cSLE care (n = 268) rated unique patient profiles; results of standard cSLE laboratory testing and information about the cSLE flare descriptors were presented as follows: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and erythrocyte sedimentation rate (ESR). Using rater interpretation of the course of cSLE (baseline versus followup as the gold standard), performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]) of the preliminary flare criteria was tested. An international consensus conference was held to rank the preliminary flare criteria as per the American College of Rheumatology recommendations and delineate threshold scores for minor, moderate, and major flares. Results: The accuracy of the 2 highest-ranked candidate criteria that consider absolute changes ((increment)) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) (numeric scoring: A = 12, B = 8, C = 1, and D/E = 0), MD-global, PCR, and ESR were confirmed (both AUC >0.93). For the SLEDAI-based criteria (0.5 × (increment)SLEDAI + 0.45 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥6.4/3.0/0.6 constituted major/moderate/minor flares, respectively. For the BILAG-based algorithm (0.4 × (increment)BILAG + 0.65 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥7.4/3.7/2.2 delineated major/moderator/minor flares, respectively. These threshold values (SLEDAI, BILAG) were all >82% sensitive and specific for capturing flare severity. Conclusion: Provisional criteria for global flares in cSLE are available to identify patients who experienced a flare. These criteria also allow for discrimination of the severity of cSLE exacerbations.

Original languageEnglish (US)
JournalArthritis Care and Research
DOIs
StateAccepted/In press - Jan 1 2018

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Systemic Lupus Erythematosus
Blood Sedimentation
Creatinine
Area Under Curve
Proteins
ROC Curve
Physicians
Sensitivity and Specificity

ASJC Scopus subject areas

  • Rheumatology

Cite this

Brunner, H. I., Holland, M., Beresford, M. W., Ardoin, S. P., Appenzeller, S., Silva, C. A., ... Ying, J. (Accepted/In press). American College of Rheumatology Provisional Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus. Arthritis Care and Research. https://doi.org/10.1002/acr.23557

American College of Rheumatology Provisional Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus. / Brunner, Hermine I.; Holland, Michael; Beresford, Michael W.; Ardoin, Stacy P.; Appenzeller, Simone; Silva, Clovis A.; Flores, Francisco; Goilav, Beatrice; Wenderfer, Scott E.; Levy, Deborah M.; Ravelli, Angelo; Khunchandani, Raju; Avcin, Tadej; Klein-Gitelman, Marisa S.; Feldman, Brian M.; Ruperto, Nicolino; Ying, Jun.

In: Arthritis Care and Research, 01.01.2018.

Research output: Contribution to journalArticle

Brunner, HI, Holland, M, Beresford, MW, Ardoin, SP, Appenzeller, S, Silva, CA, Flores, F, Goilav, B, Wenderfer, SE, Levy, DM, Ravelli, A, Khunchandani, R, Avcin, T, Klein-Gitelman, MS, Feldman, BM, Ruperto, N & Ying, J 2018, 'American College of Rheumatology Provisional Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus', Arthritis Care and Research. https://doi.org/10.1002/acr.23557
Brunner, Hermine I. ; Holland, Michael ; Beresford, Michael W. ; Ardoin, Stacy P. ; Appenzeller, Simone ; Silva, Clovis A. ; Flores, Francisco ; Goilav, Beatrice ; Wenderfer, Scott E. ; Levy, Deborah M. ; Ravelli, Angelo ; Khunchandani, Raju ; Avcin, Tadej ; Klein-Gitelman, Marisa S. ; Feldman, Brian M. ; Ruperto, Nicolino ; Ying, Jun. / American College of Rheumatology Provisional Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus. In: Arthritis Care and Research. 2018.
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title = "American College of Rheumatology Provisional Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus",
abstract = "Objective: To validate the preliminary criteria of global flare for childhood-onset SLE (cSLE). Methods: Pediatricians experienced in cSLE care (n = 268) rated unique patient profiles; results of standard cSLE laboratory testing and information about the cSLE flare descriptors were presented as follows: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and erythrocyte sedimentation rate (ESR). Using rater interpretation of the course of cSLE (baseline versus followup as the gold standard), performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]) of the preliminary flare criteria was tested. An international consensus conference was held to rank the preliminary flare criteria as per the American College of Rheumatology recommendations and delineate threshold scores for minor, moderate, and major flares. Results: The accuracy of the 2 highest-ranked candidate criteria that consider absolute changes ((increment)) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) (numeric scoring: A = 12, B = 8, C = 1, and D/E = 0), MD-global, PCR, and ESR were confirmed (both AUC >0.93). For the SLEDAI-based criteria (0.5 × (increment)SLEDAI + 0.45 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥6.4/3.0/0.6 constituted major/moderate/minor flares, respectively. For the BILAG-based algorithm (0.4 × (increment)BILAG + 0.65 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥7.4/3.7/2.2 delineated major/moderator/minor flares, respectively. These threshold values (SLEDAI, BILAG) were all >82{\%} sensitive and specific for capturing flare severity. Conclusion: Provisional criteria for global flares in cSLE are available to identify patients who experienced a flare. These criteria also allow for discrimination of the severity of cSLE exacerbations.",
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T1 - American College of Rheumatology Provisional Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus

AU - Brunner, Hermine I.

AU - Holland, Michael

AU - Beresford, Michael W.

AU - Ardoin, Stacy P.

AU - Appenzeller, Simone

AU - Silva, Clovis A.

AU - Flores, Francisco

AU - Goilav, Beatrice

AU - Wenderfer, Scott E.

AU - Levy, Deborah M.

AU - Ravelli, Angelo

AU - Khunchandani, Raju

AU - Avcin, Tadej

AU - Klein-Gitelman, Marisa S.

AU - Feldman, Brian M.

AU - Ruperto, Nicolino

AU - Ying, Jun

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Objective: To validate the preliminary criteria of global flare for childhood-onset SLE (cSLE). Methods: Pediatricians experienced in cSLE care (n = 268) rated unique patient profiles; results of standard cSLE laboratory testing and information about the cSLE flare descriptors were presented as follows: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and erythrocyte sedimentation rate (ESR). Using rater interpretation of the course of cSLE (baseline versus followup as the gold standard), performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]) of the preliminary flare criteria was tested. An international consensus conference was held to rank the preliminary flare criteria as per the American College of Rheumatology recommendations and delineate threshold scores for minor, moderate, and major flares. Results: The accuracy of the 2 highest-ranked candidate criteria that consider absolute changes ((increment)) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) (numeric scoring: A = 12, B = 8, C = 1, and D/E = 0), MD-global, PCR, and ESR were confirmed (both AUC >0.93). For the SLEDAI-based criteria (0.5 × (increment)SLEDAI + 0.45 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥6.4/3.0/0.6 constituted major/moderate/minor flares, respectively. For the BILAG-based algorithm (0.4 × (increment)BILAG + 0.65 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥7.4/3.7/2.2 delineated major/moderator/minor flares, respectively. These threshold values (SLEDAI, BILAG) were all >82% sensitive and specific for capturing flare severity. Conclusion: Provisional criteria for global flares in cSLE are available to identify patients who experienced a flare. These criteria also allow for discrimination of the severity of cSLE exacerbations.

AB - Objective: To validate the preliminary criteria of global flare for childhood-onset SLE (cSLE). Methods: Pediatricians experienced in cSLE care (n = 268) rated unique patient profiles; results of standard cSLE laboratory testing and information about the cSLE flare descriptors were presented as follows: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and erythrocyte sedimentation rate (ESR). Using rater interpretation of the course of cSLE (baseline versus followup as the gold standard), performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]) of the preliminary flare criteria was tested. An international consensus conference was held to rank the preliminary flare criteria as per the American College of Rheumatology recommendations and delineate threshold scores for minor, moderate, and major flares. Results: The accuracy of the 2 highest-ranked candidate criteria that consider absolute changes ((increment)) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) (numeric scoring: A = 12, B = 8, C = 1, and D/E = 0), MD-global, PCR, and ESR were confirmed (both AUC >0.93). For the SLEDAI-based criteria (0.5 × (increment)SLEDAI + 0.45 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥6.4/3.0/0.6 constituted major/moderate/minor flares, respectively. For the BILAG-based algorithm (0.4 × (increment)BILAG + 0.65 × (increment)PCR + 0.5 × (increment)MD-global + 0.02 × (increment)ESR) flare scores ≥7.4/3.7/2.2 delineated major/moderator/minor flares, respectively. These threshold values (SLEDAI, BILAG) were all >82% sensitive and specific for capturing flare severity. Conclusion: Provisional criteria for global flares in cSLE are available to identify patients who experienced a flare. These criteria also allow for discrimination of the severity of cSLE exacerbations.

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