Practice pattern for the use of intravenous iloprost for the treatment of peripheral vasculopathy in systemic sclerosis: A case–control study from the Italian national multicenter “SPRING” (Systemic Sclerosis Progression InvestiGation) Registry
Authors: Riccieri Valeria , Pellegrino Greta , Cipolletta Edoardo , Giuggioli Dilia , Bajocchi Gianluigi , Bellando-Randone Silvia , Dagna Lorenzo , Zanframundo Giovanni , Foti Rosario , Cacciapaglia Fabio , Cuomo Giovanna , Ariani Alarico , Rosato Edoardo , Lepri Gemma , Girelli Francesco , Zanatta Elisabetta , Bosello Silvia Laura , Cavazzana Ilaria , Ingegnoli Francesca , De Santis Maria , Murdaca Giuseppe , Abignano Giuseppina , Romeo Nicoletta , Della Rossa Alessandra , Caminiti Maurizio , Iuliano Annamaria , Ciano Giovanni , Beretta Lorenzo , Bagnato Gianluca , Lubrano Ennio , De Andres Ilenia , Giollo Alessandro , Saracco Marta , Agnes Cecilia , Lumetti Federica , Spinella Amelia , Magnani Luca , Campochiaro Corrado , De Luca Giacomo , Codullo Veronica , Visalli Elisa , Di Vico Claudio , Gigante Antonietta , Saccon Francesca , Grazia Lazzaroni Maria , Franceschini Franco , Generali Elena , Mennillo Gianna , Barsotti Simone , Pagano Mariano Giuseppa , Calabrese Francesca , Furini Federica , Vultaggio Licia , Parisi Simone , Peroni Clara Lisa , Bianchi Gerolamo , Conti Fabrizio , Cozzi Franco , D’Angelo Salvatore , Doria Andrea , Fusaro Enrico , Govoni Marcello , Guiducci Serena , Iannone Florenzo , Salvarani Carlo , Sebastiani Gian Domenico , Ferri Clodoveo , Matucci-Cerinic Marco , De Angelis Rossella 
Published: January 2024

Background: Intravenous iloprost has been widely used for the treatment of systemic sclerosis peripheral vasculopathy. No agreement has been found on the regimen and the dosage of intravenous iloprost in different scleroderma subset conditions. This study aimed to evaluate the modalities of intravenous iloprost administration within a large cohort of systemic sclerosis patients from the SPRING Registry and to identify any associated clinical-demographic, instrumental or therapeutic data. Patients and Methods: Data of systemic sclerosis patients treated with intravenous iloprost for at least 1 year (case group) were retrospectively analyzed, including different timing and duration of intravenous iloprost session, and compared with those of untreated patients (control group). Results: Out of 1895 analyzed patients, 937 (49%) received intravenous iloprost treatment, while 958 (51%) were assigned to the control group. Among cases, about 70% were treated every 4 weeks, 24% with an interval of more than 4 weeks, and only 6% of less than 4 weeks. Most patients receiving the treatment every 4 weeks, or less, underwent infusion cycle for 1 day only, while if it was scheduled with an interval of more than 4 weeks, a total number of 5 consecutive days of infusions was the preferred regimen. The comparison between the two groups revealed that patients treated with intravenous iloprost had a higher frequency of DUs (p < 0.001), pitting scars (p < 0.001), diffuse cutaneous involvement (p < 0.001), interstitial lung disease (p < 0.002), as well as higher rates of anti-topoisomerase I, “ late” scleroderma pattern at nailfold videocapillaroscopy. These findings were confirmed by multivariate analysis. Conclusion: Our data provide a picture on the Italian use of intravenous iloprost among systemic sclerosis patients and showed that it was usually employed in patients with a more aggressive spectrum of the disease. The disparity of intravenous iloprost treatment strategies in the different centers suggests the need of a rational therapeutical approach based on the clinical characteristics of different patients’ subsets.