Background Systemic Sclerosis (SSc) is an autoimmune disease with high morbididy and mortality (1). American Rheumatology Association (ARA) classification criteria of 1980 have low sensibility for early and mild disease forms (2) while the new ACR/EULAR 2013 classification criteria seem to have a greater sensibility (3).
Objectives To verify the performace of the new criteria in clinical pratice in a cohort of SSc patients referred to our center dedicated to the diagnosis and treatment of SSc.
Methods We enrolled 190 patients with a clinical diagnosis of SSc or UCTD/VEDOSS made by an expert clinician. All patients underwent clinical, laboratoristic and instrumental evaluations according to the EUSTAR and VEDOSS recommendations. Finally we applied ACR classification criteria of 1980 and the new EULAR/ACR 2013 classification criteria for SSc to all patients.
Results Among the 190 patients enrolled, 14 were males (7.3%) and 176 (92.7%)were females. One hundred six patients (71.5%) had a clinical diagnosis of SSc (50 with diffuse disease form, 86 with limited disease form) and 94 (68.1%) satisfied ACR criteria of 1980. Fifty-four patients had a clinical diagnosis of UCTD/VEDOSS (28.4%). Applying the new EULAR/ACR criteria of 2013, among the 190 patients, 152 were classified as SSc. Only 134 over 136 (98.5%) patients with clinical diagnosis of SSc and only 19 over 54 (35.1%) patients with UCTD/VEDOSS were classified as SSc according to the new criteria. Sensibility and specificity of new EULAR/ACR criteria of 2013 were 97.7% and 64.8% respectively against 69% and 96.2% obtained using the ACR criteria of 1980. Moreover patients with new SSc diagnosis seem to have a significant higher number of capillaroscopic alterations and were more frequently anti-centromere positive respect to the patients that didn't satisfy the new criteria (p<0.000001 e p=0.0001 respectively).
Conclusions Our study confirms a greater sensibility of the new EULAR/ACR 2013 criteria for SSc respect to the ACR criteria of 1980. This performance allows to better classify patients with early or mild disease forms. Moreover we can speculate that the presence of capillaroscopic alterations and anti-centromere positivity are highly suggestive for the development of an established form of SSc.
Mayes MD: Arthritis Rheum. 2003
[No authors listed]: Arthritis Rheum. 1980
van den Hoogen et al.: Arthritis Rheum. 2013
Disclosure of Interest None declared