Pulmonary hypertension and granulomatous vasculitis in sarcoidosis

Abstract

To examine the recent advancements of the epidemiology, pathophysiology, clinical characteristics, radiographic studies, diagnostic modalities, treatment, and prognosis of pulmonary hypertension in sarcoidosis. A large retrospective study found 73.8% of patients with sarcoidosis listed for transplant had pulmonary hypertension. Several other studies found pulmonary hypertension to be associated mostly with advanced sarcoidosis, although frequencies in nonfibrotic disease were not uncommon. Destruction of vasculature due to fibrotic lung disease is most likely the common cause; however, other mechanisms have been proposed. In a small study, pulmonary venous occlusive disease was observed in the explanted lungs. Several studies have found an association with pulmonary function and the incidence of pulmonary hypertension. Right heart failure was seen in 21-23% of patients. In one study, high-resolution computer tomography findings, such as presence of lymphadenopathy, opacities, and thickened bronchovascular bundles, were not significantly different. Septal lines and ground-glass opacities were found at higher frequency in sarcoidosis-associated pulmonary hypertension. Corticosteroids were effective in treating some patients with sarcoidosis-associated pulmonary hypertension. Inhaled nitric oxide, epoprostenol, and bosentan have been shown to be efficacious in a small number of patients. Pulmonary hypertension is not infrequently observed in sarcoidosis. Further studies are needed to elucidate the epidemiology, mechanisms, treatment, and significance of sarcoidosis-associated pulmonary hypertension.