Francesca Ingegnoli1, Tommaso Schioppo2, Yannick Allanore3, Roberto Caporali4, Michele Colaci5, Oliver Distler6, Daniel E Furst7, Nicolas Hunzelmann8, Florenzo Iannone9, Dinesh Khanna10, Marco Matucci-Cerinic11. 1. Division of Clinical Rheumatology, ASST Pini-CTO, Milano, Italy; Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milano, Italy. Electronic address: francesca.ingegnoli@unimi.it. 2. Division of Clinical Rheumatology, ASST Pini-CTO, Milano, Italy. 3. Paris Descartes University, Cochin Hospital, Rheumatology A department, INSERM U1016, Paris, France. 4. Division of Rheumatology, University of Pavia, IRCCS S. Matteo Foundation, Pavia, Italy. 5. Rheumatology Unit, University of Modena and Reggio Emilia, Modena, Italy. 6. Department of Rheumatology, University Hospital Zurich, Switzerland. 7. Los Angeles, USA UCLA (emeritus); University of Washington, Seattle Wash; University of Florence, Florence, Italy. 8. Department of Dermatology, University of Cologne, Cologne, Germany. 9. DETO-Section of Rheumathology, Bari, Italy. 10. Ann Arbor, University of Michigan, Ann Arbor, Michigan. 11. Department of Experimental and Clinical Medicine, University of Florence & Department of Geriatric Medicine, Division of Rheumatology AOUC, Florence, Italy.
Abstract
BACKGROUND: Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by vascular impairment, immune dysfunction and collagen deposition. Raynaud's phenomenon (RP) and digital ulcers (DU) are prominent features of SSc. Intravenous (IV) iloprost (ILO), according to the recently updated EULAR recommendations, is indicated for RP after failure of oral therapy. Moreover, IV ILO could be useful in DU healing. IV ILO is currently available mainly on the European market approved for RP secondary to SSc with 3-5 days infusion cycle. Unfortunately, data published varies regarding regimen (dosage, duration and frequency). Up to now, ILO has been studied in small cohorts of patients and in few randomized controlled trials. METHODS: A systematic review of studies on IV ILO in patients with SSc complicated by DU and RP was performed. Insufficient data were available to perform a meta-analysis according to the GRADE system. We performed a three-stage internet-based Delphi consensus exercise. RESULTS: Three major indications were identified for IV ILO usage in SSc: RP non-responsive to oral therapy, DU healing, and DU prevention. IV ILO should be administered between 0.5 and 2.0ng/kg/min according to patient tolerability with a frequency depending on the indication. CONCLUSIONS: Although these suggestions are supported by this expert group to be used in clinical setting, it will be necessary to formally validate the present suggestions in future clinical trials.
BACKGROUND:Systemic sclerosis (SSc) is an autoimmune chronic disease characterized by vascular impairment, immune dysfunction and collagen deposition. Raynaud's phenomenon (RP) and digital ulcers (DU) are prominent features of SSc. Intravenous (IV) iloprost (ILO), according to the recently updated EULAR recommendations, is indicated for RP after failure of oral therapy. Moreover, IV ILO could be useful in DU healing. IV ILO is currently available mainly on the European market approved for RP secondary to SSc with 3-5 days infusion cycle. Unfortunately, data published varies regarding regimen (dosage, duration and frequency). Up to now, ILO has been studied in small cohorts of patients and in few randomized controlled trials. METHODS: A systematic review of studies on IV ILO in patients with SSc complicated by DU and RP was performed. Insufficient data were available to perform a meta-analysis according to the GRADE system. We performed a three-stage internet-based Delphi consensus exercise. RESULTS: Three major indications were identified for IV ILO usage in SSc: RP non-responsive to oral therapy, DU healing, and DU prevention. IV ILO should be administered between 0.5 and 2.0ng/kg/min according to patient tolerability with a frequency depending on the indication. CONCLUSIONS: Although these suggestions are supported by this expert group to be used in clinical setting, it will be necessary to formally validate the present suggestions in future clinical trials.
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