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

Italian Society for Rheumatology (SIR), Clodoveo Ferri, Dilia Giuggioli, Serena Guiducci, Federica Lumetti, Gianluigi Bajocchi, Luca Magnani, Veronica Codullo, Alarico Ariani, Francesco Girelli, Valeria Riccieri, Greta Pellegrino, Silvia Bosello, Rosario Foti, Elisa Visalli, Giorgio Amato, Alessia Benenati, Giovanna Cuomo, Florenzo Iannone, Fabio CacciapagliaRossella De Angelis, Francesca Ingegnoli, Rossella Talotta, Corrado Campochiaro, Lorenzo Dagna, Giacomo De Luca, Silvia Bellando-Randone, Amelia Spinella, Giuseppe Murdaca, Nicoletta Romeo, Maria De Santis, Elena Generali, Simone Barsotti, Alessandra Della Rossa, Ilaria Cavazzana, Francesca Dall'Ara, Maria G. Lazzaroni, Franco Cozzi, Andrea Doria, Erika Pigatto, Elisabetta Zanatta, Giovanni Ciano, Lorenzo Beretta, Giuseppina Abignano, Salvatore D'Angelo, Gianna Mennillo, Gianluca Bagnato, Francesca Calabrese, Maurizio Caminiti, Giuseppa Pagano Mariano, Carlo Alberto Scirè

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)40-47
Number of pages8
JournalClinical and Experimental Rheumatology
Volume38
Issue number3
Publication statusPublished - May 1 2020

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

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