Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of scleroderma spectrum

ABSTRACT Objective. Systemic sclerosis (SSc) is a severe multiple-organ disease charac- terised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society of Rheumatology pro- moted the registry SPRING (Systemic sclerosis Progression INvestiGation). Methods. The SPRING is a multi- centre rheumatological cohort study encompassing the wide scleroder- ma spectrum, namely the primary Raynaud’s phenomenon (pRP), sus- pected secondary RP, Very Early Diag- nosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteris- tics of 2,028 Italian patients’ popula- tion at the initial phase of enrollment, mainly focusing on the cohort of 1,538 patients with definite SSc. Results. Definite SSc showed signifi- cantly higher prevalence of digital ul- cers, capillaroscopic ‘late’ pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cu- taneous subset, and anti-Scl70 sero- positivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased preva- lence of unfavorable clinico-serologi- cal features. Conclusion. Nationwide registries with suitable patients’ subsetting and follow-up studies since the prodromal phase of the disease may give us valu- able insights on the SSc natural history and main prognostic factors.