Sarcoidosis-associated pulmonary hypertension in patients with near-normal lung function

SETTING: Tertiary care medical centre in Israel. BACKGROUND: Pulmonary hypertension (PH) is a predictor of poor outcome in patients with sarcoidosis. Early diagnosis may improve outcome. OBJECTIVE: To determine factors that might contribute to the early diagnosis of PH in sarcoidosis patients with near normal lung function tests. DESIGN: Retrospective patient review. METHODS: Data from 127 patients with sarcoidosis and near normal lung function tests (forced vital capacity > 70%, forced expiratory volume in 1 second > 70% and diffusion capacity of carbon monoxide [D_LCO] > 60%), who underwent high resolution computed tomography (HRCT) scan, the 6-minute walk distance (6MWD) test and echocardiogram were analysed. Demographic, clinical and HRCT findings were compared between patients with and those without PH. RESULTS: Thirty-six patients (28.3%) had PH. Patients with PH tended to have lower D_LCO (68% ± 8 vs. 75% ± 17, P = 0.038), 6MWD (308 m ± 106 vs. 486 m ± 99, P = 0.009) and exercise saturation (91 ± 4 vs. 95 ± 3, P = 0.0001) compared to those without PH. HRCT patterns in PH showed higher frequencies of interstitial thickening (P = 0.004), ground glass appearance (P = 0.01) and fibrosis (P = 0.032). In logistic regression, only 6MWD was predictive of PH (P = 0.005, 95%CI 0.970-0.995). CONCLUSION: Physiological and radiographic characteristics appeared to differentiate patients with PH from those without. Physicians should be aware of PH in patients with sarcoidosis, even in those with near normal lung function.