Pulmonary hypertension in sarcoidosis

Pulmonary hypertension has been notreported in some patients with sarcoidosis.

We retrospectively studied 53 sarcoidosis patients with persistent dyspnea despite systemic therapy for their sarcoidosis. All patients underwent cardiac catheterization to determine pulmonary artery (PA) pressure.

Of the 53 patients, six were found to have left ventricle (LV) dysfunction, including four cases of diastolic dysfunction. Of the remaining 47 patients, 26 had a systolic PA pressure > or = 40 Torr and 25 had a mean PA pressure > or = 25 Torr. Using univariate analysis of those patients with normal LV function, echocardiography, vital capacity, and diffusion lung of carbon monoxide (D(L)co) correlated with systolic and/or mean pulmonary artery pressure. For the PA systolic, only the echocardiographic estimated PA pressure and D(L)CO % predicted remained in the multiple regression model (Coefficient of determination = 0.76, p < 0.005 for both). For the PA mean pressure, the only independent variable was the echocardiographic estimate of the PA pressure (Coefficient of determination = 0.70, p < 0.005). While echocardiography was useful in many cases, in nine cases PA pressure could not be estimated because there was no tricuspid regurgitation seen. Seven of these patients had a measured PA pressure of > or = 40 Torr. Seven patients with moderate to severe pulmonary hypertension were treated with pulmonary vasodilator therapy. Five patients experienced good clinical response.

Pulmonary hypertension was commonly found in sarcoidosis patients with persistent dyspnea. For some of these patients, treatment of the pulmonary hypertension was associated with improved clinical status.