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01-02-2013

Elevated Pulmonary Arterial Systolic Pressure in Patients with Sarcoidosis: Prevalence and Risk Factors

Authors: Aggeliki Rapti, Vasileios Kouranos, Elias Gialafos, Konstantina Aggeli, John Moyssakis, Anastasios Kallianos, Charalampos Kostopoulos, Ourania Anagnostopoulou, Petros P. Sfikakis, Athol U. Wells, George E. Tzelepis

Published in: Lung | Issue 1/2013

Abstract

Background

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) >40 mmHg. Twelve of the 37 patients agreed to undergo right heart catheterization and SRPH was confirmed in nine 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 diffusing capacity for carbon monoxide (DLCO) and age were independent determinants of SRPH. Pulmonary fibrosis and left ventricular diastolic dysfunction due to cardiac sarcoidosis or other comorbidities 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).

Conclusion

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 comorbidities are frequent pathogenic mechanisms.

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Title: Elevated Pulmonary Arterial Systolic Pressure in Patients with Sarcoidosis: Prevalence and Risk Factors
Authors: Aggeliki Rapti
Vasileios Kouranos
Elias Gialafos
Konstantina Aggeli
John Moyssakis
Anastasios Kallianos
Charalampos Kostopoulos
Ourania Anagnostopoulou
Petros P. Sfikakis
Athol U. Wells
George E. Tzelepis
Publication date: 01-02-2013
Publisher: Springer-Verlag
Published in: Lung / Issue 1/2013
Print ISSN: 0341-2040
Electronic ISSN: 1432-1750
DOI: https://doi.org/10.1007/s00408-012-9442-4

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