TY - JOUR
T1 - Systemic sclerosis Progression INvestiGation (SPRING) Italian registry
T2 - demographic and clinico-serological features of the scleroderma spectrum
AU - Italian Society for Rheumatology (SIR)
AU - Ferri, Clodoveo
AU - Giuggioli, Dilia
AU - Guiducci, Serena
AU - Lumetti, Federica
AU - Bajocchi, Gianluigi
AU - Magnani, Luca
AU - Codullo, Veronica
AU - Ariani, Alarico
AU - Girelli, Francesco
AU - Riccieri, Valeria
AU - Pellegrino, Greta
AU - Bosello, Silvia
AU - Foti, Rosario
AU - Visalli, Elisa
AU - Amato, Giorgio
AU - Benenati, Alessia
AU - Cuomo, Giovanna
AU - Iannone, Florenzo
AU - Cacciapaglia, Fabio
AU - De Angelis, Rossella
AU - Ingegnoli, Francesca
AU - Talotta, Rossella
AU - Campochiaro, Corrado
AU - Dagna, Lorenzo
AU - De Luca, Giacomo
AU - Bellando-Randone, Silvia
AU - Spinella, Amelia
AU - Murdaca, Giuseppe
AU - Romeo, Nicoletta
AU - De Santis, Maria
AU - Generali, Elena
AU - Barsotti, Simone
AU - Della Rossa, Alessandra
AU - Cavazzana, Ilaria
AU - Dall'Ara, Francesca
AU - Lazzaroni, Maria G.
AU - Cozzi, Franco
AU - Doria, Andrea
AU - Pigatto, Erika
AU - Zanatta, Elisabetta
AU - Ciano, Giovanni
AU - Beretta, Lorenzo
AU - Abignano, Giuseppina
AU - D'Angelo, Salvatore
AU - Mennillo, Gianna
AU - Bagnato, Gianluca
AU - Calabrese, Francesca
AU - Caminiti, Maurizio
AU - Pagano Mariano, Giuseppa
AU - Scirè, Carlo Alberto
PY - 2020/5/1
Y1 - 2020/5/1
N2 - OBJECTIVES: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised 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 for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation). METHODS: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc. RESULTS: Definite SSc showed a significantly higher prevalence of digital ulcers, 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 cutaneous subset, and anti-Scl70 seropositivity 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 prevalence of unfavourable clinico-serological features. CONCLUSIONS: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.
AB - OBJECTIVES: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised 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 for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation). METHODS: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc. RESULTS: Definite SSc showed a significantly higher prevalence of digital ulcers, 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 cutaneous subset, and anti-Scl70 seropositivity 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 prevalence of unfavourable clinico-serological features. CONCLUSIONS: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.
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M3 - Article
C2 - 32301427
AN - SCOPUS:85090055023
VL - 38
SP - 40
EP - 47
JO - Clinical and Experimental Rheumatology
JF - Clinical and Experimental Rheumatology
SN - 0392-856X
IS - 3
ER -