title: Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis-associated pulmonary hypertension in the PHAROS registry.
abstract: OBJECTIVE
  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).
  
  
  METHODS
  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.
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  NT-proBNP may be more useful than BNP in the detection and monitoring of PAH in SSc patients, but additional studies are necessary.
authors.affiliation: Stanford University, Stanford, CA and VA Palo Alto Health Care System, Palo Alto, CA, USA. shauwei@stanford.edu.
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
authors.affiliation: Stanford University, Stanford, CA, USA.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Fairchild
authors.affiliation: University of California Los Angeles, CA, USA.
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
authors.affiliation: Stanford University, Stanford, CA, USA.
authors.forename: Shufeng
authors.initials: S
authors.lastname: Li
authors.affiliation: University of Massachusetts Medical School, Worcester, MA/University of North Carolina-Chapel Hill, Charlotte, NC, USA.
authors.forename: Firas
authors.initials: F
authors.lastname: Alkassab
authors.affiliation: Massachusetts General Hospital, Boston, MA, USA.
authors.forename: Marcy B
authors.initials: MB
authors.lastname: Bolster
authors.affiliation: Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: Mary Ellen
authors.initials: ME
authors.lastname: Csuka
authors.affiliation: University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Chris T
authors.initials: CT
authors.lastname: Derk
authors.affiliation: University of Pittsburgh, PA, USA.
authors.forename: Robyn T
authors.initials: RT
authors.lastname: Domsic
authors.affiliation: University of Colorado School of Medicine, Aurora, CO, USA.
authors.forename: Aryeh
authors.initials: A
authors.lastname: Fischer
authors.affiliation: University of Utah, Salt Lake City, UT, USA.
authors.forename: Tracy M
authors.initials: TM
authors.lastname: Frech
authors.affiliation: University of Chicago, IL, USA.
authors.forename: Mardi
authors.initials: M
authors.lastname: Gomberg-Maitland
authors.affiliation: Hospital for Special Surgery, New York, NY, USA.
authors.forename: Jessica K
authors.initials: JK
authors.lastname: Gordon
authors.affiliation: Northwestern University, Chicago, IL, USA.
authors.forename: Monique
authors.initials: M
authors.lastname: Hinchcliff
authors.affiliation: Rutgers-RWJ Medical School, New Brunswick, NJ, USA.
authors.forename: Vivien
authors.initials: V
authors.lastname: Hsu
authors.affiliation: Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Laura K
authors.initials: LK
authors.lastname: Hummers
authors.affiliation: University of Michigan, Ann Arbor, MI, USA.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.affiliation: University of Pittsburgh, PA, USA.
authors.forename: Thomas A Jr
authors.initials: TAJ
authors.lastname: Medsger
authors.affiliation: University of Minnesota, Minneapolis, MN, USA.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Molitor
authors.affiliation: Tufts University School of Medicine, Boston, MA, USA.
authors.forename: Ivana R
authors.initials: IR
authors.lastname: Preston
authors.affiliation: University of Michigan, Ann Arbor, MI, USA.
authors.forename: Elena
authors.initials: E
authors.lastname: Schiopu
authors.affiliation: Center for Rheumatology, Albany, NY, USA.
authors.forename: Lee
authors.initials: L
authors.lastname: Shapiro
authors.affiliation: Medical University of South Carolina, Charleston, SC, USA.
authors.forename: Faye
authors.initials: F
authors.lastname: Hant
authors.affiliation: Medical University of South Carolina, Charleston, SC, USA.
authors.forename: Richard
authors.initials: R
authors.lastname: Silver
authors.affiliation: Boston University, Boston, MA, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Simms
authors.affiliation: Northwestern University, Chicago, IL, USA.
authors.forename: John
authors.initials: J
authors.lastname: Varga
authors.affiliation: Georgetown University, Washington, DC, USA.
authors.forename: Virginia D
authors.initials: VD
authors.lastname: Steen
authors.affiliation: Stanford University, Stanford, CA, and Vera Moulton Wall Center for Pulmonary Vascular Disease, USA.
authors.forename: Roham T
authors.initials: RT
authors.lastname: Zamanian
mesh_terms: D000368:Aged; D018450:Disease Progression; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000175:diagnosis* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D010446:Peptide Fragments / Q000097:blood; D011446:Prospective Studies; D012042:Registries; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications*
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain