title: Prostacyclin and oral vasodilator therapy in sarcoidosis-associated pulmonary hypertension: a retrospective case series.
abstract: BACKGROUND
  It is unclear whether recent advances in pulmonary arterial hypertension therapy can be safely applied to sarcoidosis-associated pulmonary hypertension (SAPH). Evidence for prostacyclin (PG) therapy in SAPH is limited.
  
  
  METHODS
  We conducted a single-center, retrospective review of 46 patients with sarcoidosis, 26 of whom had SAPH. Thirteen received PG as monotherapy or in combination with oral vasodilators.
  
  
  RESULTS
  Follow-up right-sided heart catheterization at a mean of 12.7 months revealed improved cardiac output, cardiac index, and pulmonary vascular resistance. Functional class and N-terminal pro-brain natriuretic peptide levels also improved in patients treated with PG. No significant change in oxygen requirement was seen with vasodilator therapy initiation. At 2 years, 15 patients with SAPH survived, including eight on PG, and at 5 years, seven survived, including five on PG. Survival was significantly reduced in patients with SAPH compared with patients who had sarcoidosis without pulmonary hypertension. Multivariate analysis demonstrated that the use of PG therapy in SAPH is not associated with increased mortality.
  
  
  CONCLUSIONS
  Many patients with severe SAPH showed significant hemodynamic and clinical improvement on long-term IV or subcutaneous PG therapy and had survival outcomes similar to patients with moderate SAPH on oral vasodilator therapy.
authors.affiliation: Section of Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL. Electronic address: catherine.bonham@uchospitals.edu.
authors.forename: Catherine A
authors.initials: CA
authors.lastname: Bonham
authors.affiliation: Section of Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL.
authors.forename: Justin M
authors.initials: JM
authors.lastname: Oldham
authors.affiliation: Section of Cardiology, University of Chicago, Chicago, IL.
authors.forename: Mardi
authors.initials: M
authors.lastname: Gomberg-Maitland
authors.affiliation: Section of Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL.
authors.forename: Rekha
authors.initials: R
authors.lastname: Vij
mesh_terms: D000284:Administration, Oral; D000959:Antihypertensive Agents / Q000008:administration & dosage; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D011464:Epoprostenol / Q000008:administration & dosage*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure / Q000502:physiology*; D012189:Retrospective Studies; D017565:Sarcoidosis, Pulmonary / Q000150:complications* / Q000503:physiopathology; D013997:Time Factors; D014655:Vascular Resistance; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D000959:Antihypertensive Agents; D014665:Vasodilator Agents; D011464:Epoprostenol