Inhaled iloprost for sarcoidosis associated pulmonary hypertension

摘要: Rationale: Patients with sarcoidosis associated pulmonary hypertension (SAPH) have responded to systemic prostacyclin therapy. Objectives: To determine the rate of response inhaled prostacyclin, iloprost, in SAPH. Methods: Sarcoidosis patients and no evidence for left ventricular dysfunction were enrolled an open label, prospective study. underwent right heart catheterization six minute walk (6MW) test. Quality life was evaluated using several instruments, including Saint George Respiratory Questionnaire (SGRQ). received 5 mcg iloprost every 2-3 hours while awake. After four months therapy, repeat cardiac catheterization, 6 MW test, completed quality questionnaires. Measurements Main Results: Of 22 enrolled, 15 all 16 weeks The most common reasons study discontinuation included drug cough (3 patients) compliance prescribed number treatments per day (2 patients). Six experienced a 20% or greater decrease vascular resistance (PVR) from baseline five these also showing 3 mm Hg reduction PA mean. Although three improved 6MW distance by at least 30 meters, only one had PVR. At significant reported SGRQ activity score (p=0.0273), seven having 4 point decrease. Conclusion: Inhaled as monotherapy improvement hemodynamics assessed some