Pulmonary hypertension (PH) remains a significant predictor of mortality in patients with advanced sarcoidosis. With the advent of newer therapies for PH and with the increasing use of lung transplantation (LT) for sarcoidosis it is important to determine if simple clinical characteristics identify subjects with sarcoidosis complicated by PH.
We reviewed the records of all patients with sarcoidosis who listed for LT in the US between Jan. 1995 and Dec. 2002 to identify those with data available from right heart catheterization. We defined PH as a mean pulmonary artery (PA) pressure of > 25 mmHg. Patients with PH were compared to those lacking PH with respect demographics, pulmonary function, functional status, need for supplemental oxygen, co-morbid illnesses, and requirement for corticosteroid therapy.
The final cohort included 363 patients of whom 73.4% had PH. In univariate analysis, neither spirometric testing (e.g., FVC, FEV1), patient age, demographic factors, nor need for corticosteroids segregated those with PH from individuals with normal PA pressures. However, persons with PH were more likely to be not working, (OR 2.2, 95% CI: 1.3-3.4) and to have systemic hypertension (OR: 2.2 95% CI: 1.1-4.2). Subjects with PH also required more supplemental oxygen (2.7±1.8 l/min vs 1.6±1.4 l/min, p=0.001). In multivariate analysis, only need for supplemental oxygen remained an independent predictor of PH –the need for supplemental oxygen approximately doubled the risk for PH (OR: 2.0. 95% CI: 1.4-2.7). As a screening test for PH, need for oxygen had a sensitivity and specificity of 91.8% and 32.6%, respectively.
PH is common in persons with sarcoidosis listed for LT. Need for supplemental oxygen is the only easily obtainable clinical variable that identifies patients with PH.
Physicians should consider more aggressive screening for PH in subjects with advanced sarcoidosis because the condition is prevalent, associated with poor outcomes, and difficult to detect based on simple clinical criteria.
A.F. Shorr, None.