| Popis: |
Sarcoidosis-associated pulmonary hypertension (SAPH) has been reported in 10% of all sarcoidosis patients. In the United States, the prevalence is similar to that reported for scleroderma. There are several possible mechanisms for SAPH, including vascular compression, granulomatous angiitis, left ventricular (LV) dysfunction, and fibrosis of lung tissue. The prognosis of those with LV dysfunction is significantly better than those with precapillary pulmonary hypertension. Screening for SAPH includes evaluation of dyspnea by 6-minute walk test, echocardiography, and measurement of pulmonary artery diameter on computer tomographic scan. Right heart catheterization remains the definitive test for characterizing SAPH. Treatment for precapillary hypertension has been shown to improve pulmonary hemodynamics. However, it is still unclear whether such treatments will change the natural course of the disease, especially in those with severe fibrosis. |
