Agreement Between Physician Evaluation and the Composite Response Index in Diffuse Cutaneous Systemic Sclerosis Conferences uri icon

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  • ObjectiveDiffuse cutaneous systemic sclerosis (SSc) is a highly heterogeneous disease. A provisionally approved Composite Response Index in diffuse cutaneous SSc (CRISS) was developed as a 1‐year outcome measure for clinical trials. Our goal was to further validate the CRISS by examining agreement between CRISS definitions for improved/non‐improved with physicians' evaluation of disease.MethodsPatient profiles from a large observational cohort were created for 50 random diffuse cutaneous SSc patients of <5 years disease duration with improved CRISS scores after 1 year and 50 with non‐improved CRISS scores. Profiles described disease features used during the initial CRISS development at baseline and at 1 year. Each profile was independently rated by 3 expert physicians. Majority opinion determined whether a patient was improved or not improved, and kappa agreement with the CRISS cutoff of 0.6 was calculated.ResultsPatients had mean ± SD disease duration of 2.2 ± 1.3 years. There was substantial agreement between the physician majority opinion about each case and the CRISS (κ = 0.76 [95% confidence interval (95% CI) 0.64–0.88]). The agreement between each individual physician opinion and the CRISS was also substantial (κ = 0.70 [95% CI 0.62–0.78]). All CRISS non‐improvers were also rated as non‐improved by physician majority; however, 12 CRISS improvers were rated as non‐improved by physicians.ConclusionThere was substantial agreement between the dichotomous CRISS rating and physician assessment of diffuse cutaneous SSc patients after 1 year. This supports the use of a CRISS cutoff at 0.6 for improvement versus non‐improvement, although the CRISS tended to rate more patients as improved than did physicians.


  • Larche, Margaret
  • Zheng, Boyang
  • Wang, Mianbo
  • McKenna, Kerry
  • Shapiro, Lee
  • Silver, Richard
  • Csuka, Mary Ellen
  • van den Hoogen, Frank
  • Robinson, David
  • Pauling, John D
  • Hummers, Laura
  • Krieg, Thomas
  • Del Galdo, Francesco
  • Spiera, Robert
  • Jones, Niall
  • Khalidi, Nader
  • Vacca, Alessandra
  • de Vries‐Bouwstra, Jeska K
  • Gordon, Jessica
  • Baron, Murray

publication date

  • November 2022