Study objectives: To determine the cause of pulmonary
hypertension (PH) in systemic sclerosis (SSc) patients since PH can
occur because of pulmonary arteriopathy, pulmonary parenchymal
destruction, and left ventricular cardiac dysfunction.
Design and setting: Consecutive case series in a university
Patients: Nine SSc patients with PH (mean
pulmonary artery pressure, 41 mm Hg), with (n = 6) or without
(n = 3) concomitant interstitial lung disease (ILD).
Methods: Acute infusion of epoprostenol was begun at 2
ng/kg/min and was titrated upward at a rate of 2 ng/kg/min every 30 min
until symptomatic complications developed or pulmonary artery vascular
resistance (PVR) was reduced by 50%.
of nine patients demonstrated a reduction of ≥ 20% in PVR,
suggesting that vasoreactivity is common despite the presence of
significant ILD. A single patient had no response to infusion with
unchanged hemodynamics and oxygenation. One patient developed hypoxemia
as cardiac output increased, suggesting a worsening of
ventilation/perfusion matching or the presence of an anatomic
shunt. Acute pulmonary edema developed in one patient at an
infusion rate of 6 ng/kg/min. The results of cardiac catheterization
suggested that pulmonary edema was caused by SSc heart disease.
Conclusion: SSc patients with ILD have diverse and
sometimes multiple causes of PH that can be determined by short-term
epoprostenol infusion. Beneficial effects can be obtained from
epoprostenol despite extensive ILD.