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

  • Simone NegriniEmail author
  • 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 Randone
  • Cosimo Bruni
  • Serena Guiducci
  • Elisabetta Battaglia
  • Maria Ilenia De Andres
  • Alessandra Azzurra Russo
  • Lorenzo Beretta
Original Article
  • 9 Downloads

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.

Keywords

Systemic sclerosis Iloprost PROSIT Vasculopathy Raynaud’s phenomenon Digital ulcers 

Abbreviations

SSc

Systemic sclerosis

RP

Raynaud phenomenon

DU

Digital ulcers

EULAR

European League Against Rheumatism

CCBs

Dihydropiridine-type calcium channel blockers

PDE5i

Phosphodiesterase type 5 inhibitors

PROSIT

Utilizzo dei PROstanoidi endovenoSI nel Trattamento della vasculopatia sclerodermica

GILS

Gruppo Italiano Lotta alla Sclerodermia

ACR

American College of Rheumatology

TSQM

Treatment Satisfaction Questionnaire for medication

ERAs

Endothelial receptor antagonists

Notes

Acknowledgements

This work was supported by Grants from Gruppo Italiano Lotta alla Sclerodermia (GILS). The sponsors had no role in study design, in the collection, analysis and interpretation of data, in the writing of the report, and in the decision to submit the article for publication.

Authors’ contributions

SN, LB, MCC, FI and OM were responsible for study conception and design, statistical analysis and interpretation of data, and writing and revising the article. All authors contributed to patients’ recruitment and data collection. All authors read and approved the final manuscript.

Compliance with ethical standards

Conflict of interest

Authors declared that they have not competing interests.

Availability of data and material

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Ethics approval and consent to participate

The study was approved by local ethics committees and conducted in accordance with the principles of the Declaration of Helsinki; all patients signed a written informed consent.

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Simone Negrini
    • 1
    Email author
  • Ottavia Magnani
    • 1
  • Marco Matucci-Cerinic
    • 2
  • Renato Carignola
    • 3
  • Valeria Data
    • 3
  • Erika Montabone
    • 3
  • Alessandro Santaniello
    • 4
  • Giuditta Adorni
    • 4
  • Giuseppe Murdaca
    • 1
  • Francesco Puppo
    • 1
  • Francesco Indiveri
    • 1
  • Alessandra Della Rossa
    • 5
  • Anna D’Ascanio
    • 5
  • Simone Barsotti
    • 5
  • Dilia Giuggioli
    • 6
  • Clodoveo Ferri
    • 6
  • Federica Lumetti
    • 6
  • Silvia Laura Bosello
    • 7
  • Giovanni Canestrari
    • 7
  • Silvia Bellando Randone
    • 2
  • Cosimo Bruni
    • 2
  • Serena Guiducci
    • 2
  • Elisabetta Battaglia
    • 8
  • Maria Ilenia De Andres
    • 8
  • Alessandra Azzurra Russo
    • 8
  • Lorenzo Beretta
    • 4
  1. 1.Department of Internal MedicineUniversity of Genoa - Policlinico San MartinoGenoaItaly
  2. 2.Department of Experimental and Clinical MedicineAOU Careggi - University of FlorenceFlorenceItaly
  3. 3.Internal MedicineSan Luigi Gonzaga Hospital OrbassanoTurinItaly
  4. 4.Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico di MilanoMilanItaly
  5. 5.Rheumatology Unit, Department of Clinical and Experimental MedicineUniversity of PisaPisaItaly
  6. 6.Rheumatology UnitUniversity of Modena and Reggio Emilia, Azienda Ospedaliero UniversitariaModenaItaly
  7. 7.Rheumatology UnitFondazione Policlinico Universitario A. Gemelli IRCCSRomeItaly
  8. 8.Rheumatology UnitARNAS GaribaldiCataniaItaly

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