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Validation of the ACR/EULAR classification criteria for systemic sclerosis in patients with early scleroderma

Rheumatology International Aug 24, 2017

Araújo FC, et al. – The authors wished to validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients with early scleroderma. They concluded that the 2013 ACR/EULAR classification criteria offered high accuracy and increased sensitivity in the classification of patients with early SSc.

  • This study enrolled 56 consecutive patients with early SSc (2001 LeRoy and Medsger criteria), 122 patients with established SSc (1980 ACR classification criteria), and 141 patients with SSc–like disorders.
  • In several subsets of patients, the diagnostic performance of the 2013 ACR/EULAR criteria was compared with the 1980 ACR criteria.
  • The performance of individual variables was also obtained.
  • Receiver operating characteristic (ROC) curves and optimal cut–off values were computed.
  • The sensitivity and specificity in the whole cohort of 178 SSc patients were 77.6 and 98.5%, respectively, using the 2013 ACR/EULAR criteria and 68.5 and 100%, respectively, using the 1980 ACR criteria.
  • 28% of the patients with early SSc met the 2013 ACR/EULAR criteria.
  • Among the patients with early SSc, 53% of those who had Raynaud’s phenomenon, abnormal capillaroscopy and positive SSc–related antibodies met the 2013 ACR/EULAR criteria.
  • 0.975 was the area under the ROC curve was (95% confidence interval 0.962–0.987).
  • The best cut–off value for the total score was ≥8 (sensitivity 82%; specificity 97.9%).
  • The individual variables with the highest specificity values were proximal skin thickening, sclerodactyly (specificity 100%), telangiectasia and SSc–related antibodies (specificity 98.6%).
  • Raynaud’s phenomenon had the best sensitivity (99.4%) but had low specificity (4.2%).

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