Epoprostenol for Treatment of Pulmonary Hypertension in Patients With Systemic Lupus Erythematosus

Pulmonary hypertension with pathologicalchanges similar to those observed in primary pulmonary hypertensionoccurs in patients with systemic lupus erythematosus (SLE). Theefficacy of chronic epoprostenol therapy in SLE has not been welldescribed. The objective of this paper is to describe our experiencewith long-term epoprostenol therapy in patients with pulmonaryhypertension associated with SLE. Case seriesof six patients with SLE and associated pulmonary hypertensionreceiving chronic treatment with epoprostenol. All 6 patients had severe pulmonary hypertension.Mean pulmonary artery pressure (mPAP) was 57 ± 9 mm Hg (mean±SD), and pulmonary vascular resistance was 14 ± 7 units beforebeginning therapy with epoprostenol. In 4 patients who underwent repeathemodynamic evaluation (9 to 16 months after starting epoprostenol), mean pulmonary artery pressure decreased by 38 ± 21% andpulmonary vascular resistance by 58 ± 12%. Clinically, allpatients improved from New York Heart Association class III or IV toclass I or II. Doses of epoprostenol ranged from 4 to 46 ng/kg/min, andthe longest duration of therapy has been 2.5 years. Side effects fromepoprostenol have not differed from those seen in patients with primarypulmonary hypertension, and except for one patient, there has been noexacerbation of SLE. Epoprostenol waseffective for the treatment of pulmonary hypertension in this smallgroup of patients with SLE. Further evaluation of epoprostenol therapyfor patients with SLE and other diseases associated with pulmonaryhypertension is warranted.