PT - JOURNAL ARTICLE AU - Elisabeth Blaja AU - Suzana Jordan AU - Carmen-Marina Mihai AU - Rucsandra Dobrota AU - Mike Oliver Becker AU - Britta Maurer AU - Marco Matucci-Cerinic AU - Oliver Distler TI - The challenge of Very Early Systemic Sclerosis: a Combination of Mild and Early Disease? AID - 10.3899/jrheum.190976 DP - 2020 Mar 15 TA - The Journal of Rheumatology PG - jrheum.190976 4099 - http://www.jrheum.org/content/early/2020/03/09/jrheum.190976.short 4100 - http://www.jrheum.org/content/early/2020/03/09/jrheum.190976.full AB - Objective To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group of patients with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological and immunological characteristics of these patients. Methods We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 ACR/EULAR nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud’s phenomenon and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold-capillaroscopy or any organ involvement characteristic for SSc). Disease duration was defined from first Raynaud`s symptom. Results One-hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with Raynaud’s phenomenon, ANA antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features like SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) patients had a disease duration of less than 5 years, as many as 29.1% (28/96) patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing, mild disease in terms of their clinical features at first presentation. Conclusion This study showed that patients with very early SSc are a mixture of patients with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.