Barnett CF, Bonura EJ, Nathan SD et al.
National Institutes of Health, Bethesda, MD, USA.
Chest 2009;135:1455–61.
Editor’s note: Pulmonary hypertension (PH) is a common and serious complication in patients with sarcoidosis, even in the absence of lung disease or pulmonary vascular inflammation. However, the prevalence and pathogenesis of PH in sarcoidosis are unclear. A number of deleterious health effects are linked to sarcoidosis-associated PH, including persistent dyspnea, reduced exercise capacity, and increased morbidity and mortality rates. Therapeutic responses to treatment with corticosteroids and immunosuppressants have been variable, but favorable outcomes have been described in a small number of cases where treatment with pulmonary arterial hypertension (PAH)-specific agents has been undertaken. In the current retrospective analysis, the investigators evaluated PAH-specific therapies in subjects with sarcoidosis and PH.