MED-22. Successful use of eporpostenol in the treatment of SLE induced pulmonary hypertension.

Intravenous prostacyclin has been shown to improve hemodynamics and quality of life and prolongs survival in patients with secondary pulmonary hypertension who are unresponsive to conventional therapy. Its cost as well as route remains a challenge. A 24-year-old African-American female presented with shortness of breath, chest tightness and dyspnea on exertion for a week. She was in respiratory distress, tachycardic, pulse oximetry was 88% on resting room air which desaturated to 39% on ambulation; 12 lead EKG showed right axis deviation, poor R wave progression and an incomplete right bundle block. CT of the chest confirmed cardiomegaly, bilateral pleural effusions and no evidence of pulmonary embolism. During the course of hospitalization it was found that these symptoms were secondary to systemic lupus syndrome, with double stranded DNA positive and ANA titers as >1:2567. ECHO revealed a right ventricular systolic pressure (RSVP) of 105 mm Hg. The patient was initially given Ca-channel blocker without any significant response therefore epoprostenol was administered incremental doses up to 14 nanogram per minute, at which pulmonary artery pressure began to decrease significantly (60/35). The systemic pressures also decreased to 99/43 mm Hg. Repeat right heart catheterization showed improvement of cardiac output, cardiac index. Right ventricular systolic pressure was 42 mm Hg, compared to previous RSVP of 105 mm Hg. This proves that Epoprostenol infusion can be effectively used in the treatment of pulmonary hypertension secondary to SLE. Review of the literature reveals that although there are few case reports of use of Epoprostenol in SLE, the efficacy of chronic use has not been well described. Severe arteriopathy of pulmonary hypertension is commonly seen in association with connective tissue disease. Further evaluation of the use of epoprostenol therapy for patients with SLE is warranted.

Fawzia K. Salahuddin, MD, and Rashid Rahman, MD. Emergency Medicine and Internal Medicine and Pulmonary and Critical Care Medicine, University of Illinois College of Medicine, Peoria, IL.