Background Previously, Early Systemic Sclerosis (Early-SSc) patients were defined as patients with Raynaud phenomenon (RP) and either a scleroderma marker autoantibody or typical capillaroscopy abnormalities or both.
Since 2013 the definition of Early-SSc has been updated: Patients should not meet the new 2013 ACR/EULAR criteria for the classification of Systemic Sclerosis (SSc). EUSTAR group also proposed preliminary criteria for the very early diagnosis of SSc which have not yet been validated.
Objectives Our aim was to revise the usefulness of Le Roy criteria and Very Early EUSTAR criteria in identifying patients at risk of development of SSc.
Methods Retrospective study of a wide and unselected series of patients with Early-SSc and SSc from a single university hospital from June 2012 to August 2015. We excluded patients who fulfilled 2013 ACR/EULAR criteria at first visit. Patients were classified as Early-SSc following Le Roy criteria and classified as SSc according to the 2013 ACR/EULAR criteria during follow-up. We reviewed EUSTAR criteria in both groups.
Results We included a total of 56 patients with a mean age of 55±15 years (94.6% women; 5.4% men). At first visit, 15 (26.8%) of our patients fulfilled 2013 ACR/EULAR criteria and were excluded of the final analysis. 37 (66.1%) of our patients fulfilled Le Roy criteria at first visit. The remaining 4 patients (7.1%) did not meet any of these criteria in the first visit. During the follow-up of this group of 41 patients, 37 (90.2%) presented RP and 9 (22%) presented puffy fingers. Antinuclear antibodies (ANA) were present in 34 (82.9%) of the patients, Anti-centromere antibodies in 24 (58.5%) patients and 29 (70.7%) patients presented pathologic capillaroscopic exam.
After a mean follow-up period of 26.1±16.6 months, 20 (48.8%) patients fulfilled 2013 ACR/EULAR criteria. The remaining 21 patients remaining classified as Early-SSc following Le Roy criteria. Of the following EUSTAR criteria, only significant positivity of ANA was observed more frequently in patients that fulfilled 2013 ACR/EULAR criteria: ANA (100%vs63.6%;p=0.005), anti-centromere antibodies (65%vs50%;p=0.41), raynaud phenomenon (95%vs85.7%;p=0.31), abnormal capillaroscopic exam (65%vs76.2%;p=0.8), presence of puffy fingers (25%vs19%;p=0.65).
Conclusions In our study, over the half of the patients initially classified as Early-SSc progressed to SSc during follow-up. EUSTAR preliminary criteria were more frequent in patients who during follow-up fulfilled 2013 ACR/EULAR criteria for SSc, although only the positivity of ANA was statistically significant. Further studies are needed to better characterize the patients with Early-SSc and Very Early-SSc.
Disclosure of Interest None declared