title: Long-term effects of intravenous iloprost in patients with idiopathic pulmonary arterial hypertension deteriorating on non-parenteral therapy.
abstract: BACKGROUND
  The majority of patients with idiopathic pulmonary arterial hypertension (IPAH) in functional classes II and III are currently being treated with non-parenteral therapies, including endothelin receptor antagonists (ERA), phosphodiesterase (PDE)-5 inhibitors, inhaled iloprost or combinations of these substances. If these treatments fail, current guidelines recommend the addition of parenteral prostanoid therapy. There is, however, limited evidence for the efficacy of parenteral prostanoids when added to combinations of non-parenteral therapies.
  
  
  METHODS
  In this retrospective, multicentre study we collected data from consecutive IPAH patients receiving intravenous iloprost in addition to optimized non-parenteral therapy between Jan 2002 and Dec 2009. Analyses included 6 min walk distance (6MWD), functional class, need for transplantation, and survival.
  
  
  RESULTS
  During the observation period, 50 patients were treated with intravenous iloprost in addition to non-parenteral therapy; 44% of the patients were on dual combination therapy and 52% on triple combination. Three months after initiation of iloprost, functional class had improved in 24% of the patients and the median 6MWD had increased from 289 m to 298 m (n.s.). During the observation period, 22 patients (44%) died and 14 (28%) underwent lung transplantation. The probabilities of LuTx-free survival at 1, 3 and 5 years following iloprost initiation were 38%, 17% and 17%, respectively. A 6MWD < 300 m and persistent functional class IV at 3 months after initiation of intravenous iloprost were predictors of an adverse outcome.
  
  
  CONCLUSIONS
  In essence, late initiation of intravenous iloprost in IPAH patients who previously failed to respond to non-parenteral therapies appears to be of limited efficacy in the majority patients. Alternative therapeutic options are currently not available, underlying the need for the development of new drugs.
authors: Department of Respiratory Medicine, Hannover Medical School, Hanover, Germany. knudsen.lars@mh-hannover.de
authors: Lars
authors: L
authors: Knudsen
authors: Alexander
authors: A
authors: Schurawlew
authors: Nils
authors: N
authors: Nickel
authors: Henning
authors: H
authors: Tiede
authors: Hossein A
authors: HA
authors: Ghofrani
authors: Heinrike
authors: H
authors: Wilkens
authors: Ralf
authors: R
authors: Ewert
authors: Michael
authors: M
authors: Halank
authors: Hans
authors: H
authors: Klose
authors: Carlos
authors: C
authors: Bäzner
authors: Jürgen
authors: J
authors: Behr
authors: Marius M
authors: MM
authors: Hoeper
mesh_terms: D000284:Administration, Oral; D000328:Adult; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D016285:Iloprost / Q000008:administration & dosage / Q000627:therapeutic use*; D007275:Injections, Intravenous; D008297:Male; D008875:Middle Aged; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D014665:Vasodilator Agents; D016285:Iloprost