Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis.

OBJECTIVE To investigate the frequency of pulmonary hypertension (PH) and clinical parameters associated with PH in sarcoidosis patients. METHODS A prospective, observational study was performed on 246 consecutive Japanese sarcoidosis patients followed up at the outpatient sarcoidosis clinic in the Central Clinic of Kyoto. The patients were evaluated for PH by Doppler echocardiography. Among these patients, 192 underwent pulmonary function tests. In addition, high-resolution CT of the lung was evaluated for the presence of lymph node enlargement, lung opacity, and thickening of bronchovascular bundles in 122 patients. PH was defined as estimated systolic pulmonary artery pressure (sPAP) > or = 40 mm Hg. The frequency of PH was evaluated, and clinical parameters were compared between patients with PH and those without PH. RESULTS Among 212 patients who were successfully evaluated for sPAP, 12 patients (5.7%) had PH. Patients with PH had the following clinical characteristics: advanced chest radiographic stage, decreased oxygen saturation, predominantly male gender, and decreased percentage of predicted vital capacity, percentage of predicted FVC, percentage of predicted FEV1, percentage of predicted functional residual capacity, and percentage of predicted total lung capacity (%TLC). Multivariate logistic regression analysis showed that decreased %TLC was independently associated with PH. There was a weak negative correlation between sPAP and %TLC (p < 0.05). CONCLUSIONS The frequency of PH in Japanese sarcoidosis patients was 5.7% evaluated with Doppler echocardiography. Decreased lung volume increases the risk of PH developing in patients with sarcoidosis.