Iloprost use and medical management of systemic sclerosis-related vasculopathy in Italian tertiary referral centers: results from the PROSIT study

Simone Negrini, Ottavia Magnani, Marco Matucci-Cerinic, Renato Carignola, Valeria Data, Erika Montabone, Alessandro Santaniello, Giuditta Adorni, Giuseppe Murdaca, Francesco Puppo, Francesco Indiveri, Alessandra Della Rossa, Anna D’Ascanio, Simone Barsotti, Dilia Giuggioli, Clodoveo Ferri, Federica Lumetti, Silvia Laura Bosello, Giovanni Canestrari, Silvia Bellando RandoneCosimo Bruni, Serena Guiducci, Elisabetta Battaglia, Maria Ilenia De Andres, Alessandra Azzurra Russo, Lorenzo Beretta

Research output: Contribution to journalArticle

Abstract

Vasculopathy is a crucial feature of systemic sclerosis (SSc), and Raynaud’s phenomenon (RP) and digital ulcers (DU) have a deep impact on the quality of patients’ life. The management of vascular disease can be challenging for the clinician because of the suboptimal tolerability of the treatments and lack of consensus on the best therapeutic approach. Intravenous iloprost, a synthetic analogue of prostacyclin, is broadly used for the treatment of RP and ischemic ulcers secondary to SSc. However, no standardized protocol on iloprost use is currently available and, consequently, the management of this treatment is largely based on the experience of each single center. The PROSIT project is an observational, multicenter study aiming to investigate the current treatments for SSc vasculopathy, the use of prostanoids, with special regard to iloprost, and the perception of the treatment from a patient’s perspective. The study was conducted on a cohort of 346 patients from eight Italian centers and included a structured survey addressed to physicians, data collected from patient’s medical records and two patient-administered questionnaires assessing the level of satisfaction, tolerability and perception of the efficacy of Iloprost. PROSIT data confirmed that in the contest of SSc iloprost represents the first-line choice for the management of severe RP and DU. Moreover, it is a well-tolerated treatment as reported by patients’ experience. Although a standard protocol for the treatment of SSc-related vasculopathy is lacking, PROSIT study identified different therapeutic approaches largely supported by tertiary Italian centers. Further studies are needed in order to optimize the best treatment for SSc vascular diseases, in particular to improve the best iloprost schedule management.

Original languageEnglish
Pages (from-to)357-366
JournalClinical and Experimental Medicine
Volume19
Issue number3
DOIs
Publication statusPublished - Jan 1 2019

Keywords

  • Digital ulcers
  • Iloprost
  • PROSIT
  • Raynaud’s phenomenon
  • Systemic sclerosis
  • Vasculopathy

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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  • Cite this

    Negrini, S., Magnani, O., Matucci-Cerinic, M., Carignola, R., Data, V., Montabone, E., Santaniello, A., Adorni, G., Murdaca, G., Puppo, F., Indiveri, F., Della Rossa, A., D’Ascanio, A., Barsotti, S., Giuggioli, D., Ferri, C., Lumetti, F., Bosello, S. L., Canestrari, G., ... Beretta, L. (2019). Iloprost use and medical management of systemic sclerosis-related vasculopathy in Italian tertiary referral centers: results from the PROSIT study. Clinical and Experimental Medicine, 19(3), 357-366. https://doi.org/10.1007/s10238-019-00553-y