title: Long-term outcomes of domiciliary intravenous iloprost in idiopathic and connective tissue disease-associated pulmonary arterial hypertension.
abstract: There are few published data on the efficacy of i.v. iloprost in pulmonary arterial hypertension (PAH). We present long-term outcomes in PAH patients receiving i.v. iloprost in a large UK referral centre.
  
  
  
  Eighty patients with idiopathic PAH (iPAH, n = 46) or PAH associated with connective tissue disease (CTD-PAH, n = 34) were identified as receiving domiciliary i.v. iloprost between January 1999 and April 2015. Baseline characteristics, doses achieved, functional class at follow-up and survival data were retrieved from hospital databases.
  
  
  
  Median maximum dose achieved was 4.6 ng/kg/min in the iPAH group and 5.0 ng/kg/min in CTD-PAH patients. Exercise capacity significantly improved in the first 6 months of therapy in IPAH patients. Overall 1-, 3- and 5-year survival was 78%, 64% and 52% in iPAH (P = 0.002) and 64%, 26% and 21% in CTD-PAH. Independent predictors of survival were age and exercise capacity.
  
  
  
  We report improved survival to that previously reported in iPAH patients treated with domiciliary i.v. iloprost. This may be in part related to higher administered doses. Patients with CTD-PAH had poorer survival, reinforcing the need for early transplantation referral in suitable patients.
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: Sheila
authors.initials: S
authors.lastname: Ramjug
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: Nehal
authors.initials: N
authors.lastname: Hussain
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: Judith
authors.initials: J
authors.lastname: Hurdman
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: Charlie A
authors.initials: CA
authors.lastname: Elliot
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.|Department of Infection and Immunity, University of Sheffield, Sheffield, UK.
authors.forename: Ian
authors.initials: I
authors.lastname: Sabroe
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: Iain J
authors.initials: IJ
authors.lastname: Armstrong
authors.affiliation: Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.|Respiratory Function Unit, Royal Hallamshire Hospital, Sheffield, UK.
authors.forename: Catherine
authors.initials: C
authors.lastname: Billings
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: Neil
authors.initials: N
authors.lastname: Hamilton
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: David G
authors.initials: DG
authors.lastname: Kiely
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, UK.|Academic Department of Respiratory Medicine, University of Sheffield, Sheffield, UK.
authors.forename: Robin
authors.initials: R
authors.lastname: Condliffe
mesh_terms: D061605:Administration, Intravenous; D000328:Adult; D003240:Connective Tissue Diseases / Q000150:complications*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage / Q000009:adverse effects; D000069451:Long Term Adverse Effects / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D016019:Survival Analysis; D016896:Treatment Outcome; D006113:United Kingdom / Q000453:epidemiology; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000009:adverse effects
chemical_list: D014665:Vasodilator Agents; D016285:Iloprost
keywords: connective tissue disease; idiopathic disease; intravenous iloprost; pulmonary arterial hypertension; survival