Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension

Sci Rep. 2019 Mar 11;9(1):4061. doi: 10.1038/s41598-019-40030-w.

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.

MeSH terms

  • Aged
  • Biomarkers / blood
  • Cardiac Catheterization
  • Echocardiography
  • Epoprostenol / administration & dosage
  • Epoprostenol / analogs & derivatives
  • Female
  • Hemodynamics / drug effects*
  • Humans
  • Iloprost / administration & dosage
  • Male
  • Middle Aged
  • Pulmonary Arterial Hypertension / blood
  • Pulmonary Arterial Hypertension / complications
  • Pulmonary Arterial Hypertension / drug therapy*
  • Pulmonary Arterial Hypertension / physiopathology
  • Sarcoidosis, Pulmonary / blood
  • Sarcoidosis, Pulmonary / complications
  • Sarcoidosis, Pulmonary / drug therapy*
  • Sarcoidosis, Pulmonary / physiopathology
  • Treatment Outcome
  • Vascular Resistance / physiology
  • Ventricular Dysfunction, Right / physiopathology

Substances

  • Biomarkers
  • Epoprostenol
  • Iloprost
  • treprostinil