Chest
Original ResearchSarcoidosis-Associated Pulmonary Hypertension: Outcome With Long-term Epoprostenol Treatment
Section snippets
Design and Data Collection
In accordance with the protocol approved by the Institutional Review Board, we retrospectively reviewed all cases of pulmonary hypertension evaluated in the Boston University Pulmonary Hypertension Center between January 2000 and October 2004 and identified patients with a concurrent diagnosis of sarcoidosis. Data collected included patient demographics, sarcoidosis stage, treatment, pulmonary function, echocardiography results, baseline and posttreatment hemodynamics, complications of
Patients
Eight patients with sarcoidosis-associated pulmonary hypertension were identified (Table 1). In 75% of cases, sarcoidosis was diagnosed based on accepted clinical findings and biopsy results demonstrating noncaseating granulomas. Two patients were considered to have sarcoidosis based on typical clinical features (hilar lymphadenopathy, upper lobe fibrosis, typical eye involvement) in the absence of biopsy. Of the five patients reported who underwent long-term epoprostenol treatment, all had
Discussion
We report a series of patients with sarcoidosis-associated pulmonary hypertension, including their clinical characteristics and their outcome with both short-term and long-term IV epoprostenol treatment. In keeping with previous reports,1, 24, 8, 14 the majority of our patients had evidence of pulmonary fibrosis and required supplemental oxygen, suggesting that pulmonary parenchymal destruction and hypoxia account, at least in part, for the observed pulmonary hypertension in these patients.
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The authors have no conflicts of interest to disclose.