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Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis-associated pulmonary hypertension in the PHAROS registry

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28


  1. Stanford University, Stanford, CA and VA Palo Alto Health Care System, Palo Alto, CA, USA.
  2. Stanford University, Stanford, CA, USA.
  3. University of California Los Angeles, CA, USA.
  4. Stanford University, Stanford, CA, USA.
  5. University of Massachusetts Medical School, Worcester, MA/University of North Carolina-Chapel Hill, Charlotte, NC, USA.
  6. Massachusetts General Hospital, Boston, MA, USA.
  7. Medical College of Wisconsin, Milwaukee, WI, USA.
  8. University of Pennsylvania, Philadelphia, PA, USA.
  9. University of Pittsburgh, PA, USA.
  10. University of Colorado School of Medicine, Aurora, CO, USA.
  11. University of Utah, Salt Lake City, UT, USA.
  12. University of Chicago, IL, USA.
  13. Hospital for Special Surgery, New York, NY, USA.
  14. Northwestern University, Chicago, IL, USA.
  15. Rutgers-RWJ Medical School, New Brunswick, NJ, USA.
  16. Johns Hopkins University, Baltimore, MD, USA.
  17. University of Michigan, Ann Arbor, MI, USA.
  18. University of Pittsburgh, PA, USA.
  19. University of Minnesota, Minneapolis, MN, USA.
  20. Tufts University School of Medicine, Boston, MA, USA.
  21. University of Michigan, Ann Arbor, MI, USA.
  22. Center for Rheumatology, Albany, NY, USA.
  23. Medical University of South Carolina, Charleston, SC, USA.
  24. Medical University of South Carolina, Charleston, SC, USA.
  25. Boston University, Boston, MA, USA.
  26. Northwestern University, Chicago, IL, USA.
  27. Georgetown University, Washington, DC, USA.
  28. Stanford University, Stanford, CA, and Vera Moulton Wall Center for Pulmonary Vascular Disease, USA.

2017 Vol.35, N°4 ,Suppl.106
PI 0106, PF 0113

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PMID: 27908301 [PubMed]

Received: 01/03/2016
Accepted : 28/09/2016
In Press: 10/11/2016
Published: 27/10/2017


To assess the utility of B-type natriuretic peptide (BNP) and N-terminal pro-BNP (NT-proBNP) in detecting and monitoring pulmonary hypertension (PH) in systemic sclerosis (SSc).
PHAROS is a multicenter prospective cohort of SSc patients at high risk for developing pulmonary arterial hypertension (SSc-AR-PAH) or with a definitive diagnosis of SSc-PH. We evaluated 1) the sensitivity and specificity of BNP≥64 and NT-proBNP≥210 pg/mL for the detection of SSc-PAH and/ or SSc-PH in the SSc-AR-PAH population; 2) baseline and longitudinal BNP and NT-proBNP levels as predictors of progression to SSc-PAH and/or SSc-PH; 3) baseline BNP≥180, NT-proBNP≥553 pg/mL, and longitudinal changes in BNP and NT-proBNP as predictors of mortality in SSc-PH diagnosed patients.
172 SSc-PH and 157 SSc-AR- PAH patients had natriuretic peptide levels available. Median BNP and NT-proBNP were significantly higher in the SSc-PH versus SSc-AR-PAH group. The sensitivity and specificity for SSc-PAH detection using baseline BNP≥64 pg/mL was 71% and 59%; and for NT-proBNP≥210 pg/mL, 73% and 78%. NT-proBNP showed stronger correlations with haemodynamic indicators of right ventricular dysfunction than BNP. Baseline creatinine, RVSP > 40 mmHg, and FVC%:DLco% ratio ≥1.8 were associated with progression from SSc-AR-PAH to SSc-PH but no association with individual or combined baseline BNP and NT-proBNP levels was observed. Baseline and follow-up BNP or NT-proBNP levels were not predictive of death, however, a composite BNP/NT-proBNP group predicted mortality (HR 3.81 (2.08-6.99), p<.0001).
NT-proBNP may be more useful than BNP in the detection and monitoring of PAH in SSc patients, but additional studies are necessary.

Rheumatology Article