title: Evidence for the Use of Epoprostenol to Treat Raynaud's Phenomenon With or Without Digital Ulcers.
abstract: To review the evidence for using intravenous (IV) epoprostenol to treat Raynaud's phenomenon (RP).
  
  
  
  The databases MEDLINE (1946 to March 2016), PubMed, and International Pharmaceutical Abstracts were searched using the terms epoprostenol, Flolan, Raynaud's disease, and CREST syndrome. Further literature sources were identified by reviewing article citations.
  
  
  
  All English-language, clinical trials and case series evaluating IV epoprostenol for the management or treatment of RP were included. Lower-quality evidence were incorporated due to limited information.
  
  
  
  Seven small uncontrolled studies/case series, 1 small placebo controlled study, and 1 larger randomized trial were identified and included. There was no consistent measurement of efficacy utilized, but improvements in hand temperature, RP attack duration and frequency were commonly associated with IV epoprostenol treatment (5 trials). There were conflicting data regarding effect sustainability, with 5 trials showing long-term effects and 3 showing immediate effects. Fewer ischemic ulcers developed during treatment with IV epoprostenol in 1 trial compared to conventional treatment. Ulcer healing ocurred in 2 trials. Common adverse effects included hypotension, headache, flushing, gastrointestinal symptoms, and jaw pain.
  
  
  
  Available evidence supports the use of IV epoprostenol for treatment of severe RP in patients refractory or intolerant to standard therapies. The dose, titration schedule, and duration of IV epoprostenol utilized in studies varied, but a conservative approach to initiation should be considered. Patients who do not respond to intermittent infusions and have severe digital ischemia may require more aggressive regimens.
authors.affiliation: Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA Joseph.Cruz@Rutgers.edu.|Department of Pharmacy, Englewood Hospital Medical Center, Englewood, NJ, USA.
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Cruz
authors.affiliation: Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA.
authors.forename: Ashley
authors.initials: A
authors.lastname: Ward
authors.affiliation: Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA.
authors.forename: Shannon
authors.initials: S
authors.lastname: Anthony
authors.affiliation: Montefiore Medical Center, Bronx, NY, USA.
authors.forename: Susanna
authors.initials: S
authors.lastname: Chang
authors.affiliation: Englewood Hospital Medical Center, Englewood, NJ, USA.
authors.forename: Hyun Billy
authors.initials: HB
authors.lastname: Bae
authors.affiliation: Ernest Mario School of Pharmacy, Rutgers, The State University of New Jersey, Piscataway, NJ, USA.|Department of Pharmacy, Robert Wood Johnson University Hospital, New Brunswick, NJ, USA.
authors.forename: Evelyn R
authors.initials: ER
authors.lastname: Hermes-DeSantis
mesh_terms: D000328:Adult; D002986:Clinical Trials as Topic; D011464:Epoprostenol / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D005385:Fingers / Q000098:blood supply*; D006801:Humans; D007262:Infusions, Intravenous; D007511:Ischemia / Q000150:complications / Q000188:drug therapy; D008875:Middle Aged; D011928:Raynaud Disease / Q000188:drug therapy* / Q000209:etiology; D014456:Ulcer / Q000150:complications / Q000188:drug therapy*
chemical_list: D011464:Epoprostenol
keywords: Flolan; PGI2; Raynaud’s disease; epoprostenol; ischemic ulcers; prostacyclin; systemic sclerosis