Literature DB >> 30989453

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

Simone Negrini1, Ottavia Magnani2, Marco Matucci-Cerinic3, Renato Carignola4, Valeria Data4, Erika Montabone4, Alessandro Santaniello5, Giuditta Adorni5, Giuseppe Murdaca2, Francesco Puppo2, Francesco Indiveri2, Alessandra Della Rossa6, Anna D'Ascanio6, Simone Barsotti6, Dilia Giuggioli7, Clodoveo Ferri7, Federica Lumetti7, Silvia Laura Bosello8, Giovanni Canestrari8, Silvia Bellando Randone3, Cosimo Bruni3, Serena Guiducci3, Elisabetta Battaglia9, Maria Ilenia De Andres9, Alessandra Azzurra Russo9, Lorenzo Beretta5.   

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.

Entities:  

Keywords:  Digital ulcers; Iloprost; PROSIT; Raynaud’s phenomenon; Systemic sclerosis; Vasculopathy

Mesh:

Substances:

Year:  2019        PMID: 30989453     DOI: 10.1007/s10238-019-00553-y

Source DB:  PubMed          Journal:  Clin Exp Med        ISSN: 1591-8890            Impact factor:   3.984


  7 in total

1.  Sudden winter iloprost withdrawal in scleroderma patients during COVID-19 pandemic.

Authors:  Enrico De Lorenzis; Gerlando Natalello; Lucrezia Verardi; Pier Giacomo Cerasuolo; Laura Gigante; Maria Antonietta D'Agostino; Silvia Laura Bosello
Journal:  Microvasc Res       Date:  2022-07-11       Impact factor: 3.750

2.  Raynaud's phenomenon.

Authors:  Ashraful Haque; Michael Hughes
Journal:  Clin Med (Lond)       Date:  2020-11       Impact factor: 2.659

Review 3.  Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review.

Authors:  Ming Zhao; Jiali Wu; Haijing Wu; Amr H Sawalha; Qianjin Lu
Journal:  Clin Rev Allergy Immunol       Date:  2021-01-15       Impact factor: 8.667

Review 4.  Management of Endothelial Dysfunction in Systemic Sclerosis: Current and Developing Strategies.

Authors:  Djúlio César Zanin-Silva; Maynara Santana-Gonçalves; Marianna Yumi Kawashima-Vasconcelos; Maria Carolina Oliveira
Journal:  Front Med (Lausanne)       Date:  2021-12-22

5.  Iloprost Duration for Digital Ulcers in Systemic Sclerosis: French Retrospective Study at Two Centers and Literature Review.

Authors:  Céline Jamart; Hervé Levesque; Sara Thietart; Olivier Fain; Sébastien Rivière; Ygal Benhamou; Arsène Mekinian
Journal:  Front Med (Lausanne)       Date:  2022-07-06

6.  [Reality of inpatient vasoactive treatment with prostacyclin derivatives in patients with acral circulation disorders due to systemic sclerosis in Germany].

Authors:  A Juche; E Siegert; U Mueller-Ladner; G Riemekasten; C Günther; I Kötter; J Henes; N Blank; R E Voll; J Ehrchen; M Schmalzing; L Susok; T Schmeiser; C Sunderkoetter; J Distler; M Worm; A Kreuter; O N Horváth; M P Schön; P Korsten; G Zeidler; C Pfeiffer; T Krieg; N Hunzelmann; P Moinzadeh
Journal:  Z Rheumatol       Date:  2020-12       Impact factor: 1.372

Review 7.  Sjögren's syndrome: a systemic autoimmune disease.

Authors:  Simone Negrini; Giacomo Emmi; Monica Greco; Matteo Borro; Federica Sardanelli; Giuseppe Murdaca; Francesco Indiveri; Francesco Puppo
Journal:  Clin Exp Med       Date:  2021-06-07       Impact factor: 3.984

  7 in total

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