Sarcoidosis-associated pulmonary hypertension : Outcome with long-term epoprostenol treatment

• Published in: Chest Vol. 130; no. 5; pp. 1481 - 1488
• Main Authors: FISHER, Kimberly A, SERLIN, David M, WILSON, Kevin C, WALTER, Robert E, BERMAN, Jeffrey S, FARBER, Harrison W
• Format: Journal Article
• Language: English
• Published: Northbrook, IL American College of Chest Physicians 01.11.2006
• Subjects: Adult; Aged; Antihypertensive Agents - adverse effects; Antihypertensive Agents - therapeutic use; Biological and medical sciences; Blood Pressure - drug effects; Blood Pressure - physiology; Cardiac catheterization; Cardiology. Vascular system; Catheters; Dose-Response Relationship, Drug; Epoprostenol - adverse effects; Epoprostenol - therapeutic use; Female; Heart; Hemodynamics; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - physiopathology; Infusions, Intravenous; Intubation; Longitudinal Studies; Lung transplants; Male; Medical sciences; Middle Aged; Mortality; Patients; Pneumology; Pulmonary arteries; Pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Retrospective Studies; Sarcoidosis; Sarcoidosis, Pulmonary - complications; Sarcoidosis, Pulmonary - physiopathology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Steroids; Treatment Outcome; Vascular Resistance - drug effects; Vascular Resistance - physiology; Veins & arteries; New York; United States > US; prostanoid therapy; Prognosis; Vasodilator agent; Prostaglandin I2; Sarcoidosis; Respiratory disease; Arachidonic acid derivatives; Cardiovascular disease; Long term; Pulmonary hypertension; Antiplatelet agent; Epoprostenol; Association; Treatment; granulomatous disease; Systemic disease; Prostanoid; Evolution; Pulmonary vascular disease; Pulmonary circulation; Prostaglandin derivatives
• ISSN: 0012-3692; 1931-3543
• DOI: 10.1378/chest.130.5.1481

Pulmonary 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. To determine the characteristics and outcome of patients with sarcoidosis-associated pulmonary hypertension treated with IV epoprostenol. Retrospective 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. Data collected included patient demographics, sarcoidosis stage, pulmonary function, echocardiography results, treatment, baseline and posttreatment hemodynamic measurements, and clinical outcome. Eight 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). In 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.