Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases

Arthritis Rheum. 1994 Oct;37(10):1528-33. doi: 10.1002/art.1780371018.


Objective: To determine the effects of short-term, maximum-tolerated-dose and long-term, optimum-dose iloprost treatment of severe pulmonary hypertension associated with systemic sclerosis (SSc) and the primary antiphospholipid syndrome (APS).

Methods: Three patients with SSc and 2 with APS who had failed to respond to oral vasodilator therapy for pulmonary hypertension were enrolled in a 32-week, open, prospective trial. Short-term infusion of maximum-tolerated doses and continuous infusion of optimum doses of iloprost were carried out following baseline cardiac catheterization. Catheterization was repeated at 2 and 32 weeks. All 5 patients completed the study and continued therapy for an average of 82 weeks (range 58-103).

Results: Acute infusion of maximum tolerated doses significantly ameliorated the cardiac index (0.92 liters/minute/m2; P < 0.01), pulmonary artery O2 saturation (10.6%; P < 0.05), and pulmonary resistance (-6.7 units; P < 0.05). After 2 weeks of continuous infusion of optimum doses, there was improvement in pulmonary resistance (> or = 16%) and pulmonary artery O2 saturation (> 30%) in the 2 patients with primary APS. After 2 and 32 weeks, the 3 SSc patients showed variable hemodynamic responses. New York Heart Association functional class and exercise tolerance improved in all patients. There was 1 episode of bacteremia, and 1 patient died after 72 weeks of study.

Conclusion: Continuous iloprost infusion may improve exercise tolerance and quality of life in patients with severe pulmonary hypertension associated with SSc and primary APS.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Adult
  • Antiphospholipid Syndrome / complications
  • Antiphospholipid Syndrome / drug therapy
  • Antiphospholipid Syndrome / physiopathology
  • Connective Tissue Diseases / complications
  • Connective Tissue Diseases / drug therapy*
  • Connective Tissue Diseases / physiopathology
  • Exercise Tolerance / drug effects
  • Female
  • Hemodynamics / drug effects
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / physiopathology
  • Iloprost / administration & dosage*
  • Iloprost / adverse effects
  • Infusions, Intravenous
  • Male
  • Middle Aged
  • Prospective Studies
  • Time Factors


  • Iloprost