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

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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.

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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

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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.

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Authors and Affiliations

  1. Department of Internal Medicine, University of Genoa - Policlinico San Martino, Genoa, Italy

    Simone Negrini, Ottavia Magnani, Giuseppe Murdaca, Francesco Puppo & Francesco Indiveri

  2. Department of Experimental and Clinical Medicine, AOU Careggi - University of Florence, Florence, Italy

    Marco Matucci-Cerinic, Silvia Bellando Randone, Cosimo Bruni & Serena Guiducci

  3. Internal Medicine, San Luigi Gonzaga Hospital Orbassano, Turin, Italy

    Renato Carignola, Valeria Data & Erika Montabone

  4. Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico di Milano, Milan, Italy

    Alessandro Santaniello, Giuditta Adorni & Lorenzo Beretta

  5. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy

    Alessandra Della Rossa, Anna D’Ascanio & Simone Barsotti

  6. Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero Universitaria, Modena, Italy

    Dilia Giuggioli, Clodoveo Ferri & Federica Lumetti

  7. Rheumatology Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy

    Silvia Laura Bosello & Giovanni Canestrari

  8. Rheumatology Unit, ARNAS Garibaldi, Catania, Italy

    Elisabetta Battaglia, Maria Ilenia De Andres & Alessandra Azzurra Russo

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  1. Simone Negrini
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  2. Ottavia Magnani
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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.

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Correspondence to Simone Negrini.

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Authors declared that they have not competing interests.

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

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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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Negrini, S., Magnani, O., Matucci-Cerinic, M. et al. Iloprost use and medical management of systemic sclerosis-related vasculopathy in Italian tertiary referral centers: results from the PROSIT study. Clin Exp Med 19, 357–366 (2019). https://doi.org/10.1007/s10238-019-00553-y

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