Registry of Sarcoidosis Associated Pulmonary Hypertension (ReSAPH) Grant uri icon

abstract

  • In sarcoidosis, respiratory failure is the major cause of death 1. Reduced lung volume is a predictor for the at risk patient 1. Among patients with low vital capacity, the presence of pulmonary hypertension and elevated right atrial pressure are the best predictors of early mortality 2;3. There have been several single case reports of pulmonary hypertension, including therapy for pulmonary sarcoidosis 4-6. There have been a limited number of prospective studies of any group of sarcoidosis patients, because of the need for catheterization to confirm the diagnosis. On the other hand, pulmonary hypertension may be relatively common in sarcoidosis patients. In a prospective study of pulmonary sarcoidosis, over 70% had significant changes in their right ventricle ejection fraction with exercise, suggesting pulmonary hypertension 7. The cause of pulmonary hypertension in sarcoidosis is multi-factorial, including diastolic dysfunction, vessel compression by adenopathy, lung destruction by fibrosis, and granulomatous vasculitis 8. With the advent of new vasodilator drugs effective for pulmonary hypertension, interest has risen in treating pulmonary hypertension due to secondary causes, including sarcoidosis 9-12. To date, studies have usually been limited to one or two centers with minimal long term follow up for patients begun on therapy 12;13. We propose to develop a multi-center registry of sarcoidosis associated pulmonary hypertension (SAPH). With this registry, we will characterize the demographics, clinical course, hemodynamics, pulmonary physiology, and disease management of sarcoidosis associated pulmonary hypertension on the United States. We will also compare these features to non-US sites.

date/time interval

  • January 2020 - November 2022