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

Lorinda Chung, Robert M. Fairchild, Daniel E. Furst, Shufeng Li, Firas Alkassab, Marcy B. Bolster, M. E. Csuka, Chris T. Derk, Robyn T. Domsic, Aryeh Fischer, Tracy M. Frech, Mardi Gomberg-Maitland, Jessica K. Gordon, Monique Hinchcliff, Vivien Hsu, Laura K. Hummers, Dinesh Khanna, Thomas A. Medsger, Jerry A. Molitor, Ioana R. PrestonElena Schiopu, Lee Shapiro, Faye Hant, Richard Silver, Robert Simms, John Varga, Virginia D. Steen, Roham T. Zamanian

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


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-ARPAH 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-ARPAH patients had natriuretic peptide levels available. Median BNP and NTproBNP 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 NTproBNP≥ 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-ARPAH to SSc-PH but no association with individual or combined baseline BNP and NT-proBNP levels was observed. Baseline and follow-up BNP or NTproBNP levels were not predictive of death, however, a composite BNP/NTproBNP group predicted mortality (HR 3.81 (2.08-6.99), p < .0001). Conclusion. NT-proBNP may be more useful than BNP in the detection and monitoring of PAH in SSc patients, but additional studies are necessary.

Original languageEnglish (US)
Pages (from-to)S106-S113
JournalClinical and Experimental Rheumatology
StatePublished - 2017
Externally publishedYes


  • BNP
  • NT-proBNP
  • Pulmonary hypertension
  • Systemic sclerosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology


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