Systemic sclerosis progression INvestiGation (SPRING) Italian registry: Demographic and clinico-serological features of the scleroderma spectrum: Clinical and Experimental Rheumatology

C. Ferri, D. Giuggioli, S. Guiducci, F. Lumetti, G. Bajocchi, L. Magnani, V. Codullo, A. Ariani, F. Girelli, V. Riccieri, G. Pellegrino, S. Bosello, R. Foti, E. Visalli, G. Amato, A. Benenati, G. Cuomo, F. Iannone, F. Cacciapaglia, R. de AngelisF. Ingegnoli, R. Talotta, C. Campochiaro, L. Dagna, G. de Luca, S. Bellando-Randone, A. Spinella, G. Murdaca, N. Romeo, M. de Santis, E. Generali, S. Barsotti, A. della Rossa, I. Cavazzana, F. Dall’Ara, M.G. Lazzaroni, F. Cozzi, A. Doria, E. Pigatto, E. Zanatta, G. Ciano, L. Beretta, G. Abignano, S. D’Angelo, G.A. Mennillo, G. Bagnato, F. Calabrese, M. Caminiti, G. Pagano Mariano, E. Battaglia, E. Lubrano, G. Zanframundo, A. Iuliano, F. Furini, A. Zanetti, G. Carrara, F. Rumi, C.A. Scirè, M. Matucci-Cerinic, Italian Society for Rheumatology

Research output: Contribution to journalArticlepeer-review


Objective. 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 multicentre 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. Conclusion. 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. © Copyright CLINICAL AND EXPERIMENTAL RHEUMATOLOGY 2020.
Original languageEnglish
Pages (from-to)S40-S47
JournalClin. Exp. Rheumatol.
Publication statusPublished - 2020


  • Raynaud’s phenomenon
  • Systemic sclerosis
  • scl 70 antibody
  • adult
  • Article
  • capillaroscopy
  • clinical feature
  • cohort analysis
  • demography
  • disease classification
  • disease duration
  • female
  • finger ulcer
  • follow up
  • human
  • major clinical study
  • male
  • middle aged
  • population research
  • prevalence
  • primary Raynaud phenomenon
  • priority journal
  • prognosis
  • register
  • risk factor
  • scleroderma
  • secondary Raynaud phenomenon
  • sex difference
  • systemic sclerosis
  • angioscopy
  • Italy
  • Raynaud phenomenon
  • Cohort Studies
  • Humans
  • Male
  • Microscopic Angioscopy
  • Raynaud Disease
  • Registries
  • Scleroderma, Systemic


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