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Clinical aspects

 

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

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59

 

  1. University of Modena and Reggio Emilia, Italy. clferri@unimore.it
  2. University of Modena and Reggio Emilia, Italy.
  3. University of Florence, Italy.
  4. University of Modena and Reggio Emilia, Italy.
  5. Arcispedale S. Maria Nuova, Reggio Emilia, Italy.
  6. Arcispedale S. Maria Nuova, Reggio Emilia, Italy.
  7. Policlinico San Matteo, Pavia, Italy.
  8. AOU Parma, Italy.
  9. Ospedale GB Morgagni, Forlì, Italy.
  10. Università di Roma La Sapienza, Italy.
  11. Università di Roma La Sapienza, Italy.
  12. Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, UOC di Reumatologia, Roma, Italy.
  13. AOU Policlinico Vittorio Emanuele, Catania, Italy.
  14. AOU Policlinico Vittorio Emanuele, Catania, Italy.
  15. AOU Policlinico Vittorio Emanuele, Catania, Italy.
  16. AOU Policlinico Vittorio Emanuele, Catania, Italy.
  17. Università degli Studi della Campania Luigi Vanvitelli, Napoli, Italy.
  18. UO Reumatologia, DETO, Università di Bari, Italy.
  19. UO Reumatologia, DETO, Università di Bari, Italy.
  20. Clinica Reumatologica, Università Politecnica delle Marche, Ancona, Italy.
  21. Università degli Studi di Milano, Italy.
  22. Ospedale L. Sacco, Milano, Italy.
  23. Ospedale S. Raffaele, Milano, Italy.
  24. Ospedale S. Raffaele, Milano, Italy.
  25. Ospedale S. Raffaele, Milano, Italy.
  26. Università di Firenze, Italy.
  27. University of Modena and Reggio Emilia, Italy.
  28. Ospedale Policlinico S. Martino, Università di Genova, Italy.
  29. ASO S. Croce e Carle, Cuneo, Italy.
  30. Istituto Clinico Humanitas, Rozzano, Milano, Italy.
  31. Istituto Clinico Humanitas, Rozzano, Milano, Italy.
  32. AOU Santa Chiara, Università di Pisa, Italy.
  33. AOU Santa Chiara, Università di Pisa, Italy.
  34. Spedali Civili di Brescia, Italy.
  35. Spedali Civili di Brescia, and General Medicine Unit, Ospedale Maggiore di Lodi, Italy.
  36. Spedali Civili and University of Brescia, Italy.
  37. Università degli Studi di Padova, Italy.
  38. Università degli Studi di Padova, Italy.
  39. Università degli Studi di Padova, Italy.
  40. Università degli Studi di Padova, Italy.
  41. ASL Avellino, Italy.
  42. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
  43. AOR San Carlo di Potenza, Italy.
  44. AOR San Carlo di Potenza, Italy.
  45. AOR San Carlo di Potenza, Italy.
  46. Università degli Studi di Messina, Italy.
  47. SSD Reumatologia, Reggio Calabria, Italy.
  48. SSD Reumatologia, Reggio Calabria, Italy.
  49. SSD Reumatologia, Reggio Calabria, Italy.
  50. AO ARNAS Garibaldi, Catania, Italy.
  51. Università del Molise, Campobasso, Italy.
  52. Policlinico San Matteo, Pavia, Italy.
  53. AO San Camillo Forlanini, Roma, Italy.
  54. Department of Medical Sciences, University of Ferrara, Italy.
  55. Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
  56. Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
  57. Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
  58. Department of Medical Sciences, University of Ferrara, and Epidemiology Unit, Italian Society for Rheumatology, Milan, Italy.
  59. University of Florence, Italy.

on behalf of the Italian Society for Rheumatology (SIR)

CER12769
2020 Vol.38, N°3 ,Suppl.125
PI 0040, PF 0047
Clinical aspects

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PMID: 32301427 [PubMed]

Received: 13/09/2019
Accepted : 03/12/2019
In Press: 14/04/2020
Published: 26/08/2020

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.

Rheumatology Article