• Lorinda Chung, Robert M Fairchild, Daniel E Furst, Shufeng Li, Firas Alkassab, Marcy B Bolster, Mary Ellen 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 Jr Medsger, Jerry A Molitor, Ivana R Preston, Elena Schiopu, Lee Shapiro, Faye Hant, Richard Silver, Robert Simms, John Varga, Virginia D Steen, and Roham T Zamanian.
• Stanford University, Stanford, CA and VA Palo Alto Health Care System, Palo Alto, CA, USA. shauwei@stanford.edu.
• Clin Exp Rheumatol. 2017 Sep 1; 35 Suppl 106 (4): 106-113.

ObjectivesTo 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).MethodsPHAROS 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.Results172 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).ConclusionsNT-proBNP may be more useful than BNP in the detection and monitoring of PAH in SSc patients, but additional studies are necessary.

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