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

Author:

Riccieri Valeria1,Pellegrino Greta12ORCID,Cipolletta Edoardo3,Giuggioli Dilia4ORCID,Bajocchi Gianluigi5,Bellando-Randone Silvia6,Dagna Lorenzo7,Zanframundo Giovanni8ORCID,Foti Rosario9,Cacciapaglia Fabio10,Cuomo Giovanna11ORCID,Ariani Alarico12,Rosato Edoardo13,Lepri Gemma6ORCID,Girelli Francesco14,Zanatta Elisabetta15,Bosello Silvia Laura16,Cavazzana Ilaria17,Ingegnoli Francesca18,De Santis Maria19ORCID,Murdaca Giuseppe20ORCID,Abignano Giuseppina21,Romeo Nicoletta22,Della Rossa Alessandra23,Caminiti Maurizio24,Iuliano Annamaria25,Ciano Giovanni26,Beretta Lorenzo27,Bagnato Gianluca28,Lubrano Ennio29ORCID,De Andres Ilenia30,Giollo Alessandro31,Saracco Marta32,Agnes Cecilia33,Lumetti Federica4,Spinella Amelia4,Magnani Luca5,Campochiaro Corrado7,De Luca Giacomo7ORCID,Codullo Veronica8,Visalli Elisa9,Di Vico Claudio11,Gigante Antonietta13,Saccon Francesca34,Grazia Lazzaroni Maria17,Franceschini Franco17,Generali Elena19,Mennillo Gianna21,Barsotti Simone23,Pagano Mariano Giuseppa24,Calabrese Francesca24,Furini Federica35,Vultaggio Licia35,Parisi Simone36,Peroni Clara Lisa36,Bianchi Gerolamo37,Conti Fabrizio1,Cozzi Franco34,D’Angelo Salvatore21ORCID,Doria Andrea15,Fusaro Enrico36,Govoni Marcello35,Guiducci Serena6,Iannone Florenzo10ORCID,Salvarani Carlo4,Sebastiani Gian Domenico25,Ferri Clodoveo4,Matucci-Cerinic Marco6,De Angelis Rossella3

Affiliation:

1. Department of Internal Medicine, Anesthesiology and Cardiovascular Sciences, Sapienza University of Rome, Rome, Italy

2. Department of Rheumatology, IRCCS Ospedale Galeazzi–Sant’Ambrogio, Milan, Italy

3. Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy

4. Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy

5. Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy

6. Department of Experimental and Clinical Medicine, Division of Rheumatology, AOUC, University of Florence, Florence, Italy

7. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milano, Italy

8. Department of Rheumatology, Policlinico San Matteo, Pavia, Italy

9. Rheumatology Unit, A.O.U Policlinico S. Marco, Catania, Italy

10. Rheumatology Unit, Department of Precision and Regenerative Medicine-Ionian Area, University of Bari “Aldo Moro,” Bari, Italy

11. Department of Precision Medicine, University of Campania “Luigi Vanvitelli,” Naples, Italy

12. Department of Medicine, Internal Medicine and Rheumatology, Azienda Ospedaliero Universitaria di Parma, Parma, Italy

13. Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy

14. Department of Medicine, Rheumatology Unit, Ospedale GB Morgagni—L Pierantoni, Forlì, Italy

15. Rheumatology Unit, Department of Medicine (DIMED), University of Padua, Padua, Italy

16. Institute of Rheumatology and Affine Sciences, Division of Rheumatology, Catholic University of the Sacred Heart, Rome, Italy

17. Rheumatology and Clinical Immunology, Department of Clinical and Experimental Sciences, ASST Spedali Civili of Brescia and University of Brescia, Brescia, Italy

18. Division of Clinical Rheumatology, ASST Pini, Department of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan, Italy

19. Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy

20. Department of Internal Medicine, University of Genoa, IRCCS Ospedale Policlinico San Martino, Genoa, Italy

21. Rheumatology Institute of Lucania (IReL) and Rheumatology Department of Lucania, San Carlo Hospital, Potenza, Italy

22. Rheumatology Unit, ASO Santa Croce e Carle, Cuneo, Italy

23. Department of Rheumatology, University of Pisa, Pisa, Italy

24. Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria, Italy

25. Rheumatology Unit, San Camillo Forlanini Hospital, Rome, Italy

26. Hospital of Ariano Irpino, Local Health Department, Ariano Irpino, Italy

27. Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy

28. Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy

29. Department of Rheumatology, University of Molise, Campobasso, Italy

30. Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione “Garibaldi,” Catania, Italy

31. Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy

32. Ospedale Mauriziano, Torino, Italy

33. San Lorenzo Hospital, Turin, Italy

34. Department of Medicine, Villa Salus Hospital, Venice, Italy

35. Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara, Italy

36. Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino, Turin, Italy

37. Rheumatology Unit, Department of Musculoskeletal Sciences, Local Health Trust 3, La Colletta Hospital, Genoa, Italy

Abstract

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.

Publisher

SAGE Publications

Subject

Immunology,Rheumatology,Immunology and Allergy

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