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Extended report
Validation of the systemic lupus erythematosus responder index for use in juvenile-onset systemic lupus erythematosus
  1. Rina Mina1,
  2. Marisa S Klein-Gitelman2,
  3. Shannen Nelson1,
  4. B Anne Eberhard3,
  5. Gloria Higgins4,
  6. Nora G Singer5,
  7. Karen Onel6,
  8. Lori Tucker7,
  9. Kathleen M O'Neil8,
  10. Marilynn Punaro9,
  11. Deborah M Levy10,
  12. Kathleen Haines11,
  13. Alberto Martini12,
  14. Nicolino Ruperto13,
  15. Daniel Lovell1,
  16. Hermine I Brunner1
  1. 1Division of Rheumatology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA
  2. 2Division of Pediatric Rheumatology, Children's Memorial Hospital, Chicago, Illinois, USA
  3. 3Division of Pediatric Rheumatology, Steven and Alexandra Cohen Children's Medical Center of New York, New York, New York, USA
  4. 4Division of Pediatric Rheumatology, Nationwide Children's Hospital and Ohio State University, Columbus, Ohio, USA
  5. 5Division of Rheumatology, MetroHealth Medical Center and Case Western Reserve University, Cleveland, Ohio, USA
  6. 6Division of Pediatric Rheumatology, University of Chicago Comer Children's Hospital, Chicago, Illinois, USA
  7. 7Division of Pediatric Rheumatology, BC Children's Hospital, Vancouver, British Columbia, Canada
  8. 8Section of Rheumatology, Riley Hospital for Children, Indianapolis, Indiana, USA
  9. 9Division of Pediatric Rheumatology, Texas Scottish Rite Hospital, Dallas, Texas, USA
  10. 10Division of Rheumatology, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
  11. 11Section of Pediatric Rheumatology and Immunology, Joseph M Sanzari Children's Hospital, Hackensack UMC, Hackensack, New Jersey, USA
  12. 12Istituto G. Gaslini, Pediatria II, Reumatologia and Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy
  13. 13Istituto G. Gaslini, Pediatria II, Reumatologia, Genova, Italy
  1. Correspondence to Dr Rina Mina, Division of Rheumatology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH 45208, USA; rina.mina{at}


Objectives This study tested the concurrent validity of the systemic lupus erythematosus responder index (SRI) in assessing improvement in juvenile-onset systemic lupus erythematosus (jSLE).

Methods The SRI considers changes in the SELENA–SLEDAI, BILAG and a 3-cm visual analogue scale of physician-rated disease activity (PGA) to determine patient improvement. Using prospectively collected data from 760 unique follow-up visit intervals of 274 jSLE patients, we assessed the sensitivity and specificity of the SRI using these external standards: physician-rated improvement (MD-change), patient/parent-rated major improvement of wellbeing (patient-change) and decrease in prescribed systemic corticosteroids (steroid-change). Modifications of the SRI that considered different thresholds for the SELENA–SLEDAI, BILAG and 10-cm PGA were explored and agreement with the American College of Rheumatology/PRINTO provisional criteria for improvement of jSLE (PCI) was examined.

Results The sensitivity/specificity in capturing major improvement by the MD-change were 78%/76% for the SRI and 83%/78% for the PCI, respectively. There was fair agreement between the SRI and PCI (kappa=0.35, 95% CI 0.02 to 0.73) in capturing major improvement by the MD-change. Select modified versions of the SRI had improved accuracy overall. All improvement criteria tested had lower sensitivity when considering patient-change and steroid-change as external standards compared to MD-change.

Conclusions The SRI and its modified versions based on meaningful changes in jSLE have high specificity but at most modest sensitivity for capturing jSLE improvement. When used as an endpoint of clinical trials in jSLE, the SRI will provide a conservative estimate regarding the efficacy of the therapeutic agent under investigation.

  • Systemic Lupus Erythematosus
  • Epidemiology
  • Disease Activity

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