SESSION TITLE: ILD Posters
SESSION TYPE: Poster Presentations
PRESENTED ON: Saturday, March 22, 2014 at 01:15 PM - 02:15 PM
PURPOSE: Sarcoidosis-related pulmonary hypertension (SRPH) is an entity associated with significant morbidity and mortality irrespective of disease severity, while the pathogenic mechanisms remain poorly understood.
METHODS: This cross-sectional study included consecutive patients with biopsy proven sarcoidosis (n=313) who were followed up in an outpatient setting from October 2002 through June 2010. All patients underwent clinical and cardiopulmonary evaluation including cardiac MRI to assess prevalence of SRPH and identify possible underlying pathophysiological mechanisms.
RESULTS: By Doppler echocardiographic criteria, 37 (11.8%) patients were found to have pulmonary arterial systolic pressure (PASP) > 40mmHg. Twelve of the 37 patients accepted to undergo right heart catheterization and SRPH was confirmed in 9 patients. Compared to patients without SRPH, those with SRPH were significantly older, and had greater lung function impairment; disease duration did not differ between patients with and without SRPH. Multiple logistic regression analysis showed that DLCO and age were independent determinants of SRPH. Pulmonary fibrosis and left ventricular diastolic dysfunction due to cardiac sarcoidosis or other comorbities accounted for SRPH in the majority of patients. In the nonpulmonary fibrosis group, DLCO ≤50.65 (% predicted) was associated with SRPH (sensitivity 77.8%, specificity 72.2%. p=0.031, AUC= 0.759)
CONCLUSIONS: In a large cohort of sarcoidosis patients, this study found a prevalence of SRPH of about 12%. Pulmonary fibrosis and left ventricular diastolic dysfunction due to cardiac sarcoidosis or other comorbidies are frequent pathogenic mechanisms.
CLINICAL IMPLICATIONS: Sarcoidosis patients should be screened for cardiac sarcoidosis and sarcoidosis related pulmonary hypertension upon admission to the outpatient clinics.
DISCLOSURE: The following authors have nothing to disclose: Vasileios Kouranos, Aggeliki Rapti, Elias Gialafos, Konstantina Aggeli, Petros Sfikakis, Nikolaos Koulouris, Athol Wells, George Tzelepis
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