title: Efficacy and Safety of Inhaled Iloprost in Japanese Patients With Pulmonary Arterial Hypertension　- Insights From the IBUKI and AIR Studies.
abstract: BACKGROUND
  Inhaled iloprost is approved for pulmonary arterial hypertension (PAH) in many countries. IBUKI was a phase III, non-randomized, open-label study of the efficacy and safety of inhaled iloprost in Japanese patients with PAH.
  
  
  RESULTS
  Adults with PAH who were treatment-naïve or administered endothelin receptor antagonists (ERAs) and/or phosphodiesterase type 5 inhibitors (PDE5-Is) and in NYHA/WHO functional class (FC) III/IV had inhaled iloprost (2.5 µg, increased to 5.0 µg if tolerated) 6-9 times daily for 12 weeks. Eligible patients entered a 40-week extension phase. Endpoints included change from baseline to week 12 in pulmonary vascular resistance (PVR; primary endpoint), other efficacy parameters, and safety. Data were compared with new subgroup analyses of treatment-naïve Western PAH patients from the global phase III AIR study. 27 patients received iloprost: 89% were treated with an ERA and/or PDE5-I; 70% with both. At week 12, PVR improved from baseline by -124 dyn·sec·cm(-5)(95% CI, -177 to -72) and 6-min walking distance increased by 36.0 m (95% CI, 14.9 to 57.1). NYHA/WHO FC improved in 62%; none worsened. Common drug-related adverse events were headache (37%) and cough (15%); 1 patient experienced hypotension; none reported syncope or hemoptysis. There were no deaths and no unexpected long-term safety findings. AIR PAH subgroup analyses showed similar results.
  
  
  CONCLUSIONS
  Inhaled iloprost appeared effective and safe in Japanese PAH patients, including ERA- and PDE5-I-treated patients, consistent with findings of the AIR PAH subpopulation and previous iloprost studies.
authors.affiliation: Department of Pediatrics, Toho University Omori Medical Center.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Saji
authors.forename: Masafumi
authors.initials: M
authors.lastname: Myoishi
authors.forename: Koichiro
authors.initials: K
authors.lastname: Sugimura
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Tahara
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Takeda
authors.forename: Keiichi
authors.initials: K
authors.lastname: Fukuda
authors.forename: Horst
authors.initials: H
authors.lastname: Olschewski
authors.forename: Yoshimi
authors.initials: Y
authors.lastname: Matsuda
authors.forename: Sylvia
authors.initials: S
authors.lastname: Nikkho
authors.forename: Toru
authors.initials: T
authors.lastname: Satoh
mesh_terms: D000280:Administration, Inhalation; D000328:Adult; D044466:Asian People; D065128:Endothelin Receptor Antagonists / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage* / Q000009:adverse effects; D007564:Japan; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D016285:Iloprost