Sarcoidosis-Associated Pulmonary Hypertension : Outcome With Long-term Epoprostenol Treatment

RationalePulmonary hypertension is a known complication of sarcoidosis and is associated with increased mortality. Little is known about the outcome of sarcoidosis-associated pulmonary hypertension, including response to treatment.ObjectiveTo determine the characteristics and outcome of patients with sarcoidosis-associated pulmonary hypertension treated with IV epoprostenol.DesignRetrospective chart review of all cases of pulmonary hypertension with a concomitant diagnosis of sarcoidosis evaluated in the Boston University Pulmonary Hypertension Center from 2000 to 2004.MeasurementsData collected included patient demographics, sarcoidosis stage, pulmonary function, echocardiography results, treatment, baseline and posttreatment hemodynamic measurements, and clinical outcome.ResultsEight patients were identified; four of the patients had stage IV pulmonary sarcoidosis. Pulmonary function test results were notable for severe diffusion impairment (mean diffusion capacity of the lung for carbon monoxide, 30% of predicted), with only mild-to-moderate restrictive physiology (mean FVC, 59% of predicted). Seventy-five percent of patients required supplemental oxygen at the time of presentation. All patients had moderate or severe pulmonary hypertension and were New York Heart Association (NYHA)/World Health Organization (WHO) class III or IV. A vasodilator trial with epoprostenol was performed in seven of the eight patients; six of the seven patients had a significant hemodynamic response (> 25% reduction in pulmonary vascular resistance). All but one of the responders (five of six patients) continued on therapy. Average clinical improvement was one to two NYHA/WHO classes at a mean follow-up of 29 months (range, 15 to 49 months).ConclusionsIn patients with sarcoidosis-associated pulmonary hypertension, the severity of pulmonary vascular disease occurs out of proportion to lung function abnormalities. The majority of our patients responded to epoprostenol; survival may be improved in this group.