Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus

Objective: Pulmonary hypertension with pathological changes similar to thos e observed in primary pulmonary hypertension occurs in patients with system ic lupus erythematosus (SLE). The efficacy of chronic epoprostenol therapy in SLE has not been well described, The objective of this paper is to descr ibe our experience with long-term epoprostenol therapy in patients with pul monary hypertension associated with SLE. Design: Case series of sis patients with SLE and associated pulmonary hyper tension receiving chronic treatment with epoprostenol Results: All 6 patients had severe pulmonary hypertension. Mean pulmonary a rtery pressure (mPAP) was 57 +/- 9 mm Hg (mean +/- SD), and pulmonary vascu lar resistance was 14 +/- 7 units before beginning therapy with epoprosteno l, In 4 patients who underwent repeat hemodynamic evaluation (9 to 16 month s after starting epoprostenol), mean pulmonary artery pressure decreased by 38 +/- 21% and pulmonary vascular resistance by 58 +/- 12%. Clinically all patients improved from New York Heart Association class III or IV to class I or II. Doses of epoprostenol ranged from 4 to 46 ng/kg/min, and the long est duration of therapy has been 2.5 years. Side effects from epoprostenol hale not differed from those seen in patients with primary pulmonary hypert ension, and except for one patient, there has been no exacerbation of SLE, Conclusion: Epoprostenol was effective for the treatment of pulmonary hyper tension in this small group of patients with SLE, Further evaluation of epo prostenol therapy for patients with SLE and other diseases associated with pulmonary hypertension is warranted.