New criteria improved diagnoses for patients with systemic sclerosis

Revised systemic sclerosis classification criteria codeveloped by the European League Against Rheumatism and the American College of Rheumatology resulted in improved sensitivity in identifying patients with the disease, according to a study presented at the EULAR annual congress in Madrid.

“The complexity and severity of systemic sclerosis [SSc] is a growing burden on health care systems,” EULAR President Maxime Dougados, MD, rheumatology professor, René Descartes University, Cochin Hospital, in Paris, said in a press release. “Using up-to-date recommendations to accurately diagnose patients will allow them to properly heal.”

The guidelines, which replaced criteria established by the American Rheumatism Association (ARA) in 1980, were designed to address patients with early SSc and others with limited cutaneous SSc (lcSSc). Researcher prospectively examined 20 cases across the SSc spectrum and assigned weights to the disease criteria after reducing the number of items. One item was specifically identified — skin thickening of the fingers of both hands that extended proximal to the metacarpophalangeal joints — and weighted the heaviest (9 points) as an indication of SSc. The new criteria were later simplified and reclassified with seven other items to analyze a validation cohort of 405 patients. Using nine points or more as the definition of systemic sclerosis, the new guidelines displayed a sensitivity of 91% and a specificity of 92% in the cohort compared with rates of 75% and 72%, respectively, using ARA standards.

In applying the new EULAR/ACR classifications in a clinical prospective, observational study of 321 patients with SSc, including lcSSc (n=255) and diffuse SSc (n=66) patients, researchers at University Hospital Zurich assessed that 162 fulfilled ARA criteria and EULAR/ACR guidelines. Among the remaining 159 patients who did not meet ARA classification, 52 fulfilled the new criteria. The data resulted in an SSc sensitivity of 67% for patients using the EULAR/ACR classification as opposed to 51% based on ARA guidelines.

“The new EULAR/ACR classification criteria performed with a higher sensitivity and allowed more patients to be classified as definite SSc patients than the old ARA criteria,” the researchers concluded.

Disclosure: See the abstract for a full list of relevant disclosures.

For more information:

Van Den Hoogen F. OP0033: Classification Criteria for Systemic Sclerosis: Preliminary Results. Presented at: EULAR 2013; June 12-15, Madrid.

Jordan S. OP0034: Performance of the New EULAR/ACR Classification Criteria for Systemic Sclerosis in Clinical Practice. Presented at: EULAR 2013; June 12-15, Madrid.