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.
Highlights
Sarcoidosis is a systemic disease that affects the parenchyma, interstitium, thoracic lymph nodes, airways and vasculature of the lungs[1]
Www.nature.com/scientificreports cohort of patients with newly diagnosed sarcoidosis-associated pulmonary hypertension (SAPH), factors that led to treatment with pulmonary hypertension (PH)-specific therapy, and phenotypes associated with adverse clinical outcomes in follow-up
We identified 95 patients (76% female; 86% African American) with histopathological diagnosis of pulmonary sarcoidosis and new diagnosis of SAPH between 1990–2010 (Table 1)
Summary
Sarcoidosis is a systemic disease that affects the parenchyma, interstitium, thoracic lymph nodes, airways and vasculature of the lungs[1]. The presence of sarcoidosis-associated pulmonary hypertension (SAPH), classified as World Health Organization group 5 due to its complex/multifactorial mechanisms[6], is known to worsen outcomes[5,7,8]. No pulmonary arterial hypertension (PH)-specific therapies are currently approved to treat SAPH5. Prior pilot studies and anecdotal experiences have yielded overall neutral results with pulmonary vasodilators in management of patients with SAPH1,9–13. Comprehensive description of pulmonary hypertension, including hemodynamics, functional and echocardiographic findings, in SAPH is limited, especially description of real-world experiences and in context of ongoing treatment for sarcoidosis[14,15]. Www.nature.com/scientificreports cohort of patients with newly diagnosed SAPH, factors that led to treatment with PH-specific therapy, and phenotypes associated with adverse clinical outcomes in follow-up. Because of the questionable validity of 6-minute walk distance (6MWD) as a surrogate marker for PH severity in the setting of pulmonary sarcoidosis[16], we included natriuretic peptide endpoints in our study
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