Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension.
Abstract
The presence of pulmonary hypertension (PH) significantly worsens outcomes in patients
with advanced sarcoidosis, but its optimal management is unknown. We aimed to characterize
a large sarcoidosis-associated pulmonary hypertension (SAPH) cohort to better understand
patient characteristics, clinical outcomes, and management strategies including treatment
with PH therapies. Patients at Duke University Medical Center with biopsy-proven sarcoidosis
and SAPH confirmed by right heart catheterization (RHC) were identified from 1990-2010.
Subjects were followed for up to 11 years and assessed for differences by treatment
strategy for their SAPH, including those who were not treated with PH-specific therapies.
Our primary outcomes of interest were change in 6-minute walk distance (6MWD) and
change in N-terminal pro-brain natriuretic peptide (NT-proBNP) by after therapy. We
included 95 patients (76% women, 86% African American) with SAPH. Overall, 70% of
patients had stage IV pulmonary sarcoidosis, and 77% had functional class III/IV symptoms.
Median NT-proBNP value was elevated (910 pg/mL), and right ventricular dysfunction
was moderate/severe in 55% of patients. Median values for mean pulmonary artery pressure
(49 mmHg) and pulmonary vascular resistance (8.5 Woods units) were consistent with
severe pulmonary hypertension. The mortality rate over median 3-year follow-up was
32%. Those who experienced a clinical event and those who did not had similar overall
echocardiographic findings, hemodynamics, 6MWD and NT-proBNP at baseline, and unadjusted
analysis showed that only follow-up NT-proBNP was associated with all-cause hospitalization
or mortality. A sign test to evaluate the difference between NT-Pro-BNP before and
after PH therapy produced evidence that a significant difference existed between the
median pre- and post-NT-Pro-BNP (-387.0 (IQR: -1373.0-109), p = 0.0495). Use of PH-specific
therapy may be helpful in selected patients with SAPH and pre-capillary pulmonary
vascular disease. Prospective trials are needed to characterize responses to PH-specific
therapy in this subset of patients with SAPH.
Type
Journal articleSubject
Science & TechnologyMultidisciplinary Sciences
Science & Technology - Other Topics
SURVIVAL
THERAPY
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https://hdl.handle.net/10161/18198Published Version (Please cite this version)
10.1038/s41598-019-40030-wPublication Info
Parikh, Kishan S; Dahhan, Talal; Nicholl, Leigh; Ruopp, Nicole; Pomann, Gina-Maria;
Fortin, Terry; ... Rajagopal, Sudarshan (2019). Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension.
Scientific reports, 9(1). pp. 4061. 10.1038/s41598-019-40030-w. Retrieved from https://hdl.handle.net/10161/18198.This is constructed from limited available data and may be imprecise. To cite this
article, please review & use the official citation provided by the journal.
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Show full item recordScholars@Duke
Talal I Dahhan
Assistant Professor of Medicine
An Internist, Pulmonologist and Critical Care Medicine physician with great interest
in diagnosis and management of pulmonary vascular disease, as well as innovations
in critical care curricular designs and graduate medical education.
Terry Ann Fortin
Associate Professor of Medicine
Kishan S Parikh
Assistant Professor of Medicine
Duke University Medical CenterDuke Clinical Research Institute
Gina-Maria Pomann
Assistant Professor of Biostatistics & Bioinformatics
My primary research focuses on methods related to the development of biostatistics,
bioinformatics, and data science units within academic health centers. An essential
element of my work is to improve and diversify the workforce of collaborative biostatisticians
and data scientists. I develop training programs, operational processes, and organizational
infrastructure that foster efficient and effective collaborations between clinical
and translational scientists and quantitative scientists.<b
Sudarshan Rajagopal
Associate Professor of Medicine
I am a physician-scientist with a research focus on G protein-coupled receptor signaling
in inflammation and vascular disease and a clinical focus on pulmonary vascular disease,
as I serve as Co-Director of the Duke Pulmonary Vascular Disease Center. My research
spans the spectrum from clinical research in pulmonary vascular disease, to translational
research in cardiovascular disease, to the basic science of receptor signaling.
Our basic science resesarch focuses on understandin
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