Objective: Pulmonary hypertension with pathological
changes similar to those observed in primary pulmonary hypertension
occurs in patients with systemic lupus erythematosus (SLE). The
efficacy of chronic epoprostenol therapy in SLE has not been well
described. The objective of this paper is to describe our experience
with long-term epoprostenol therapy in patients with pulmonary
hypertension associated with SLE.
Design: Case series
of six patients with SLE and associated pulmonary hypertension
receiving chronic treatment with epoprostenol.
Results: 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 before
beginning therapy with epoprostenol. In 4 patients who underwent repeat
hemodynamic evaluation (9 to 16 months 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 longest duration of therapy has been 2.5 years. Side effects from
epoprostenol have not differed from those seen in patients with primary
pulmonary hypertension, and except for one patient, there has been no
exacerbation of SLE.
Conclusion: Epoprostenol was
effective for the treatment of pulmonary hypertension in this small
group of patients with SLE. Further evaluation of epoprostenol therapy
for patients with SLE and other diseases associated with pulmonary
hypertension is warranted.